SKBB II Novita Assa

EVIDENCE BASED CASE
ACUTE LYMPHOBLASTIC LEUKEMIA (L2) HIGH

RISK (C91.0), HYPERLEUKOCYTOSIS (D72.8), AND
WELL NOURISHED
By:
Novita Purnamasari Assa
POSTGRADUATED PEDIATRIC TRAINING PROGRAM

DEPARTMENT OF CHILD HEALTH
FACULTY OF MEDICINE UDAYANA UNIVERSITY
SANGLAH GENERAL HOSPITAL DENPASAR
2018
I. Identity
1. Patient identity
Name : IKH
Age on case determination : 15 years 2 months old
Date of birth : February 14th, 2003
Sex : Male
Address : Indonesia
Date of admission : April 26th, 2018
Date of case determination : May 4th, 2018
Medical record number : 18017727
2. Parents identity
Father Mother
Name (initial) IWB NNY
Age 58 years old 35 years old
Education Senior high school Senior high school
Occupation Retired Housewife
II. History Taking (Subjective)

History of illness was collected from patient and the parents.
1. History of present illness
Chief complaint: pain in the left and right waist
The patient was referred from B general hospital with suspicion of
hematological malignancy. Pain in the left and right waist complained
about one week before admission. The pain initially disappeared but was
heavier from 3 days before entering the hospital. Pain is felt more severe
on the left waist.
Patients noticed abdominal enlargement started a week before
admission. Abdomen was getting bigger and harder especially in the left
upper abdomen. Patients occasionally felt that his stomach is full,
especially after eating. Abdominal enlargement was not accompanied by
pain, nausea or vomiting. He never been constipated for a long time.
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Patients also complained of lumps in the neck, armpits and in the
inguinal from 1 month before admission. The size of the lump is about 2-
6 cm, initially on the left neck, no pain, no redness.
There was bone pain in both legs one week before admission. The
pain cannot be described by the patient, occurred especially at night and
resolved without any medication. No swelling, or previous trauma.
Other complaints such as pale, fever, pain on both eyes, blurred
vision, headache, sweating at night were denied. Abnormal eating habits
such as eating soil, paper, and others were also denied.
Appetite and activity began to decline since the illness occured.
Appetite did not improve with changes in the types of food. There was no
progressive weight loss before the illness.
Patient defecated once every day, in yellow color with solid
consistency, no dark or bloody feces. History of defecation accompanied
by itching or worms out of anal area during night was denied. Urin was
normal, clear yellow-color, not red, and not foamy. Pain during urination
was denied.
2. History of past illness
The patient was taken to the general practitioners twice due to his pain,
one week before admission. He was given paracetamol tablet and pain
killer. His symptoms did not resolve, so that the parents decided to go to
hospital.
There was no history of similar symptoms or severe disease before,
no history of prolonged bleeding after injury or invasive procedure such
as vaccine injection, no history of recurrent fever, allergy or rash. No
history of prolonged medication or routine drug consumption such as
corticosteroids or other drugs, no history of abuse or domestic violence.
No history of travel to a malaria endemic region.
3. Family medical history
Patients is the first child of the second wife. Patients have four siblings; all
siblings were in healthy condition. Mother was the first child from five
siblings, mother’s siblings was healthy. Mother suffered systemic lupus
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erythematosus since 10 years ago and until now is doing treatment. Father
was the first child from seven siblings, father’s sibling was healthy. Father
had diabetes mellitus since 15 years ago. Grandfather is said to have
leukemia at the age of 70 years and has died 20 years ago. There is no
history of allergy, tuberculosis infection on his family. Father and older
brother is a smoker. There was no history of long term exposure of
pesticides and X-ray. History of living near high voltage power lines or
mobile phone transmitter was denied.
4. Social history
a. Pre-natal history
The patient was born from a 20 years old mother with full term
pregnancy. His mother had a routine antenatal care with a midwife for
more than four times during pregnancy. His mother only took vitamin
supplements without any other medications. She had normal blood
pressure and blood sugar. There was no history of X-ray exposure,
fever, rash, pain, edema, and other illness during pregnancy, and she
had normal blood pressure.
Conclusion: normal pregnancy.
b. Intra-natal history
The Patient was born spontaneously at the hospital, assisted by a doctor.
He was vigorous with body weight was 3000 grams. There was no
history of dyspnea, cyanotic, and jaundice reported.
Conclusion: normal spontaneous delivery.
c. Postnatal history
Patient was breastfed immediately after birth. No history of
hospitalization because of jaundice, seizure, or bleeding event were
reported.
Conclusion: normal postnatal period.
d. Nutritional history
Patient were breastfed exclusively for 6 months and continued until 24
months. Patient started to consume soft food from the age of 6 months,
steamed rice since the age 9 months, and adult food since 12 months
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old. Patient’s daily food combination contains of rice, white meat, fish,
eggs, fruits, and vegetables. No food allergy was reported. Before
admission, patient consumed porridge three times daily, 3 portions of
chicken eggs, and 2 portion of vegetables soup. Total calory and
protein intake for 24 hours were 1600 kcal and protein 40 grams. Its
fulfilled energy estimated 70% from the recommended dietary
allowanced (RDA).
Conclusion: decreased food intake history after symptoms appear.
e. Growth and development history
Patient’s body weight increased every month during the first year of
life. No history of weight loss was noted. His latest body weight was
unknown. Data of his birth length was unavailable. His current height
is 166 cm. History of his development was normal or similar as his
sibling since birth until now. Patient is 15 years 2 months old now; he
is at the second year of junior high school and his development was in
accordance with his peers.
Conclusion: growth and development were appropriate for his age.
f. Immunization history
His parents could not recall precisely types and frequency of
immunizations that the patient received. Patient’s parents said that
patient had the vaccination in accordance with the immunization
schedule set by ministry of health until nine months. They also stated
that patient also had another three immunizations in elementary school.
BCG scar was found on the patient’s right arm. Patient was given
immunization at primary healthcare and school.
Conclusion: immunization was in accordance with health ministry
immunization programme.
g. Basic needs history
Stimulation : he receives sufficient stimulation from his parents. He is
able to get along with his peers.
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Parenting : he is the third child in the family and was born from a
happy marriage. He was loved and supported by both
parents.
Caring : he was breastfed exclusively for six months and
continued until 24 months. Health care was received
from a midwife or doctor from primary health care and
hospital. He lives with his parents and sibling in a
permanent house.
Conclusion : basic needs history was fulfilled.
h. Family socio-economic condition
He lived with his parents and siblings in a permanent house located in
the countryside. The house has enough ventilation. It consists of four
rooms, 10 x 6 m2 in space and a terrace. Kitchen and toilet are located
inside the house. The house is not located near high voltage power lines
or mobile phone transmitter. His father was retired from private
employee 3 years ago and his mother is a housewife. His older sibling
work as private employee. Average family income is 3,000,000 IDR
per month. The source of water comes from a well and the lighting
source comes from the state electricity company (PLN). The family has
a good relationship with the surrounding neighbors. During health
treatment, patient’s medical bills was covered by government health
insurance.
Conclusion: low-moderate socioeconomic level, the patient has public
health insurance.
III. History During Admission Until Determination as Case

On the first day of hospitalization (April 26, 2018), the patient still have
pain in the left and right waist. On physical examination, he appeared moderately
ill and alert, pulse rate was 98 beats/minute, regular with good pulse quality,
respiratory rate was 24 times/minute, regular, axillary temperature was 36.5oC, and
pain score (NRS) was 2 (mild pain). Multiple, soft, painless, enlarged lymph nodes
were palpable in the left and right pre-auricula, retro-auricula, submandibula, neck,
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axilla and inguinal, with diameters of 1-6 cm. The liver was palpable 2 cm below
the xiphoid process and 2 cm below the costal arch, with sharp edges, soft
consistency, smooth surface, and was painless. The spleen was palpable at
Schuffner V. No palpable testicular mass was found. His body weight (BW) was
51 kg, body height (BH) was 166 cm, mid upper arm circumference (MUAC) was
24 cm, standard MUAC was 26.4 cm, nutritional status based on the arm
circumference was 90% (well nourished). Laboratory findings on Mei 26th, 2018
revealed WBC 174.6 K/µL (neutrophils 14.57 K/µL (8.35%), lymphocyte 146.2
K/µL (81.21%)), hemoglobin 9.93 g/dL (MCV 84.67 fL, MCH 27.6 pg, MCHC
32.59 g/dL), hematocrit 30.45%, platelet 47.61 K/µL, corrected reticulocyte 2 %,
BUN 11.6 mg/dL, creatinine 0.72 mg/dL, natrium 138 mmol/L, kalium 4.14
mmol/L, chloride 96.8 mmol/L, calcium 8.8 mg/dL, magnesium 2.19 mg/dL,
phosphor anorganik 4.54 mg/dL, aspartate aminotransferase (AST) 21.1 U/L;
alanine aminotransferase (ALT) 22.70 U/L. Peripheral blood smear result showed
erythrocytes was normochromic anisositosis, normoblast; leukocytes was elevated,
diff. lymphocytosis, found in immature cells with a low core and cytoplasmic ratios,
without granules, as much as 20%, no toxic granules, no vacuolization;
thrombocytes was decrease, impression of suspected acute leukemia (ALL
differential diagnosis AML), suggestion for bone marrow aspiration. Urinalysis:
colorless, specific gravity 1.018, pH 6.0, leukocytes 1/HPF, erythrocytes 2/HPF.
He was diagnosed hyperleukocytosis (D72.8), bicytopenia and organomegaly et
causa suspect acute leukemia (acute lymphoblastic leukemia (C91.0)
differential diagnosis acute myeloblastic leukemia (C92.0)), differential
diagnosis limphoma non-hodgin (C85.80) and well nourished. His maintenance
fluid requirements were 2000 ml/m2/day (hydration during hyperleukocytosis),
3060 ml/day with D5 ½NS with additional 25 meq sodium bicarbonate in each 500
ml infusion bag. Calorie and protein requirements were 2295 kcal/day and 40
grams/day respectively (divided into three times of meals and snacks two times
daily). The patient was given allopurinol 10 mg/kg/day equal to 200 mg every 8
hours orally, and paracetamol 500 mg every 4 hours orally if pain scale more than
4. He was planned for bone marrow aspiration (BMA) and monitored for vital signs,
fluid balance sign of leukostasis and tumor lysis syndrome. Evaluation of urine
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potential of hydrogen (pH) was repeated every 6 hours (if pH <6.5 give sodium
bicarbonate 1.5 tablets oral), complete blood count and urinalysis repeated every
day, electrolytes and uric acid test repeated every 3 days.
On the second to fifth day of hospitalization (April 27-30, 2018), pain in the
left and right waist decreased. On physical examination, he appeared moderately ill
and alert, pulse rate was 80-90 beats/minute, regular with good pulse quality,
respiratory rate was 20-24 times/minute, regular, highest axillary temperature was
37.0oC, oxygen saturation was 98%, pain score (NHS) was 2 (mild pain). There
was multiple enlarged lymph nodes, hepatomegaly and splenomegaly (schuffner
V). Results of serial blood tests after hydration obtained WBC still high (170.20
K/µL; 145.90 K/µL; 175.9 K/µL; 174.60 K/µL), hemoglobin (9.87 g/dL, 9.52 g/dL,
8.90 g/dL, 8.67 g/dL), platelet (48.88 K/µL, 41.70 K/µL, 37.21 K/µL, 42.00). Blood
urea nitrogen was 10.20 mg/dL, creatinine 0.69 mg/dL. Electrolytes analysis results
were: sodium 139 mmol/L; potassium 4.14 mmol/L; chloride 97.2 mmol/L; calcium
8.8 mg/dL. Urinalysis: colorless, specific gravity 1.006, pH 8.0, leukocytes 1/HPF,
erythrocytes 1/HPF. He was diagnosed hyperleukocytosis (D72.8), bicytopenia
and organomegaly et causa suspect acute leukemia (acute lymphoblastic
leukemia (C91.0) differential diagnosis acute myeloblastic leukemia (C92.0)),
differential diagnosis limphoma non-hodgin (C85.80) and well nourished. His
maintenance fluid requirements were 2000 ml/m2/day (hydration during
hyperleukocytosis), 3060 ml/day with D5 ½NS with additional 25 meq sodium
bicarbonate in each 500 ml infusion bag. Calorie and protein requirements were
2295 kcal/day and 40 grams/day respectively (divided into three times of meals and
snacks two times daily). Allopurinol 10 mg/kg/day equal to 200 mg every 8 hours
stopped, and still continue paracetamol 500 mg every 4 hours orally if pain scale
more than 4. Consult anesthesia for accompaniment of anesthesia during BMA.
Patient was planned for BMA evaluation at April 30th, 2018. Vital signs, fluid
balance, sign of leukostasis and tumor lysis syndrome was monitored. Evaluation
of pH every 6 hours (if pH <6.5 give sodium bicarbonate 1.5 tablets oral), complete
blood count and urinalysis repeated every day, electrolytes and uric acid test
repeated every 3 days.
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On the sixth to eighth day of hospitalization (May 1-3, 2018), pain in the
left and right waist decreased. On physical examination, he appeared moderately ill
and alert, pulse rate was 80-90 beats/minute, regular with good pulse quality,
respiratory rate was 20-24 times/minute, regular, highest axillary temperature was
37.0oC, oxygen saturation was 98%, pain score (NHS) was 2 (mild pain). There
was multiple enlarged lymph nodes, hepatomegaly and splenomegaly (schuffner
V). Results of serial blood tests after hydration WBC (186.10 K/µL; 181.30 K/µL),
hemoglobin (7.75 g/dL, 8.24 g/dL), platelet (31.99 K/µL, 35.36 K/µL). Blood urea
nitrogen was 13.60 mg/dL, creatinine 0.83 mg/dL. Electrolytes analysis results
were: sodium 139 mmol/L; potassium 4.14 mmol/L; chloride 97.1 mmol/L; calcium
9.0 mg/dL. He was diagnosed hyperleukocytosis (D72.8), bicytopenia and
organomegaly et causa suspect acute leukemia (acute lymphoblastic leukemia
(C91.0) differential diagnosis acute myeloblastic leukemia (C92.0)),
differential diagnosis limphoma non-hodgin (C85.80) and well nourished.
Management of hyperleukocytosis such as hydration, sodium bicarbonate, and
periodic laboratory examination were continued. Vital signs, fluid balance, sign of
leukostasis and tumor lysis syndrome was monitored.
IV. Physical Examination (Objective) on May 4, 2018

a. Present status
General condition : moderately ill
Level of consciousness : E4V5M6 15/15 (Compos mentis)
Blood pressure : 110/70 mmHg (50-90th percentile)
Pulse rate : 98 beats/minutes, regular, sufficient
volume
Respiration rate : 24 times/minutes, regular
Oxygen saturation : 98% in room air
Axila temperature : 37.2°C
Pain scale (NRS) :2
b. General status
Head : normocephaly, black hair, not easily plucked.
Face : no abnormality, no edema, no syndromic facies.
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Eye : no pale conjunctiva, no icteric sclera, no sunken eyes, no
palpebral edema, symmetrical eyelids, round pupils with
both diameter 3 mm and good light reflexes, no deviation
conjugae, no strabismus, no exophthalmos.
Ear : normal shape, no discharge.
Nose : excoriation was visible on nasal septum, no nasal flare, no
discharge, no bleeding, no septal deviation.
Throat : no hyperemic in pharynx and tonsil T1/T1 no hyperemic.
Mouth : no pale, no cyanosis on surrounding mouth and tongue,
symmetrical corners of the mouth, no drooling, no tongue
enlargement, no white plaque on tongue and mouth, no
caries dentis.
Neck : multiple enlarged lymph nodes in left and right pre-
auricula, retro-auricula, submandibular, cervical anterior
and posterior, supraclavicular with diameters of 1-6 cm,
consistency of solid, flat surface, not felt warm, soft and
painless.
Axilla : multiple enlarged lymph nodes with diameters of 1-3 cm,
painless.
Chest
Heart :
Inspection : no visible precordial bulging and ictus cordis.
Palpation : ictus cordis was palpable in the intersection of left
midclavicular line and fourth intercostals space without
thrill, LV lift and RV heave not palpable.
Auscultation : normal heart sounds, regular, M1>T1, A2>P2, no
murmur.
Lung :
Inspection : normal chest shaped, symmetrical on static and dynamic
state, without retraction.
Palpation : symmetrical chest movement.
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Auscultation : vesicular breath sound, without rales or wheezing on both
lungs.
Abdomen :
Inspection : no distention, superficial vein was not seen.
Auscultation : normal peristaltic sound.
Palpation : palpable liver 2 cm below the xiphoid process and 2 cm
below the costal arch, with sharp edges, soft consistency,
smooth surface, and painless. Palpable spleen at Schuffner
V.
Percussion : tympanic, shifting dullness not found.
Limbs :
Upper : no edema, no cyanosis on fingers, palms were not pale,
warm on palpation, capillary refill time 1 second.
Lower : no edema, no cyanosis on fingers, palms were not pale,
warm on palpation, capillary refill time 1 second.
Inguinal : multiple enlarged lymph nodes with diameters of 2-3 cm,
painless.
Skin : no pale, no cutis marmorata or maculopapular rash.
Genital : no hyperemia of penis, no phimosis, no smegma, both
testicle were palpable with normal size and volume. No
mass on both testicle.
Neurological examination of four limbs:

Upper limbs Lower limbs
Strength 555 555
Tonus Normal Normal
Tropik Normal Normal
Physiological reflex Normal Normal
Pathological reflex Negative Negative
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c. Pubertal status
Gonad : enlargement of penis; further growth of testes.
Pubic hair : hair spread sparsely over the pubic region, increase in
amount and curling.
Appropriate with G3P3 pubertal status according to Tanner stage.
d. Anthropometric status (based on WHO growth chart 2006)

BW, weight for age : 51 kg, 25th-50th percentile (normal).
BH, height for age : 166 cm25th-50th percentile (normal).
Weight for height : 25th percentile (normal).
IBW : 51 kg.
MUAC : 24.0 cm.
Standard MUAC : 26.4 cm.
Nutritional status (MUAC) : 90% (well nourished).
Father height : 163 cm.
Mother height : 160 cm.
Genetic height potential : 159.5 – 176.5 cm (body height is within
genetic height potential).
5. Developmental status
PedsQL score based on parental report over the last month was 70% and
score based on child report over the last month was 75%. Pediatric
symptom checklist (PSC)-17 score was 1.
Conclusion: there is no impairment in quality of life and no behavior
problems of the patient.
6. Laboratory and radiology findings

a. Complete blood count: WBC 209.80 K/µL (neutrophil 14.74 K/µL
(7.03%); lymphocyte 178.20 K/µL (84.95%)), hemoglobin 8.03 g/dL
(MCV 88.53 fL; MCH 28.61 pg; MCHC 32.32 g/dL), hematocrit
24.84%, platelet 35.33 K/µL.
b. Bone marrow aspiration: hyperseluler, decreased erythroid activity,
decreased myeloid activity, decreased megakaryosit activity
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lymphoblast cell infiltration 70%, size varies. Conclusions: appropriate
to Acute Lymphoblastic Leukemia (ALL-L2).
c. Chest X-ray: in the normal limit.
V. Resume
A 15 years 2 months old boy was B general hospital with suspicion of
hematological malignancy. Pain in the left and right waist complained about
one week before admission. The pain initially disappeared but was heavier
from 3 days before entering the hospital. Pain is felt more severe on the left
waist. Abdominal enlargement started a week before admission. Abdomen
was getting bigger and harder especially in the left upper abdomen. Patients
occasionally felt that his stomach is full, especially after eating. Patients also
complained of lumps in the neck, armpits and in the inguinal from 1 month
before admission. The size of the lump is about 2-6 cm, initially on the left
neck, no pain, no redness. Bone pain in both legs one week before admission.
The pain cannot be described by the patient, occurred especially at night and
resolved without any medication. No swelling, or previous trauma.
On physical examination, he appeared moderately ill and alert, pulse
rate was 98 beats/minute, respiratory rate was 24 times/minute, axillary
temperature was 36.8oC. multiple enlarged lymph nodes in left and right pre-
auricula, retro-auricula, submandibular, cervical anterior and posterior,
supraclavicular, axilla, inguinal with diameters of 1-6 cm, consistency of
solid, flat surface, not felt warm, soft and painless. The liver was palpable 2
cm below the xiphoid process and 2 cm below the costal arch, with sharp
edges, soft consistency, smooth surface, and was painless. The spleen was
palpable at Schuffner V. Nutritional status based on MUAC was 90% (well
nourished). Laboratory findings revealed severe hyperleukocytosis, mild-
moderate anemia and thrombocytopenia. Blood smear: immature cells with
a low core and cytoplasmic ratios, without granules 20% suspected acute
leukemia (ALL differential diagnosis AML). Result of bone marrow
aspiration revealed hypercellular, decreased erythroid system, decreased
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myeloid system, decreased megacaryocyte system, lymphoblast infiltration
70%, with varies size, suitable for ALL L2.
VI. Diagnosis
Acute lymphoblastic leukemia (L2) high risk (C91.0), hyperleukocytosis
(D72.8), and well nourished.
VII. Problems
1. Problems risk factors
Can exposure of cigarette smoke be a risk factor for ALL compared to
without exposure of cigarette smoke?
2. Problems on management
Patients with hyperleukocytosis is a serious oncologic problem and at
risk of tumor lysis syndrome and leukostasis. After hydration, the
number of leukocytes are still high. What is complications and outcomes
of pediatric patients with hyperleukocytic acute lymphoblastic leukemia
during induction therapy?
3. Problems on prognosis
a. How is prognostic and what factors affect the prognosis and survival
among children with acute lymphoblastic leukemia?
b. How is the quality of life of children with acute lymphoblastic
leukemia?
Some of these issues will be solved by journal searching based on evidence
based practice (EBP).
VIII. Planning
1. Medical treatment plan
a. Emergency management
- Hydration of 2000 ml/m2/day equal to IVFD D5 ½NS 3060 ml/day,
adding sodium bicarbonate 25 meq in each 500 ml D5 ½ NS.
- Allopurinol 10 mg/kg/day equal to 200 mg every 8 hours orally.
- Sodium bicarbonate 1.5 tablets oral if pH urine <6.5
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b. Diagnostic investigation
- Complete blood count test will be repeated every three days.
Liver function test, kidney function test, and electrolytes test
will be repeated every two weeks during chemotherapy.
- Echocardiography will be performed before administration of
daunorubicin chemotherapy to evaluate the presence of
cardiomyopathy.
- Bone marrow aspiration will be evaluated after completion of
induction phase chemotherapy to evaluate therapy.
c. Supportive therapy
- Supportive therapy to maintenance optimal condition of the
patient including nutritional therapy and blood component
transfusion as indicated.
d. Pharmacological therapy
- Chemotherapy will be given based on Indonesian Childhood ALL-
2013 High Risk Protocol with methotrexate intrathecal and high
dose, vincristine, dexamethasone, daunorubicin, cyclophosphamide
cytarabin, L-asparginase, and 6-mercaptopurine.
2. Pediatric nutrition care
a. Nutritional assessment: well nourished.
b. Nutritional requirement: maintenance fluid requirements for the patient
is 2120 ml/day. Calorie requirements according to RDA are 45
kcal/kg/day equivalent to 2295 kcal/day, protein requirements are 0.8
g/kg/day equivalent to 40.8 grams/day.
c. Nutritional route: orally.
d. Nutritional selection: rice and side dishes, one portion every eight hours
with snacks every 12 hours.
e. Nutritional monitoring: intake, vomiting, diarrhea, mid upper arm
circumference and weight gain.
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3. Monitoring
a. Due to long term chemotherapy, about 2-3 years, patient’s adherence
and compliance are essential. Patient requires monitoring of short-term
and long-term side effects of chemotherapy.
b. Patients with ALL highly susceptible to infection. Parent’s participation
is very important to prevent infection through a healthy lifestyle,
environmental hygiene or individual hygiene.
c. Assessment of the quality of life of children and possible behavioral
disturbances was conducted with PedsQL examination.
4. Communication, information and education plan
a. The disease and management
Communication, information and education about the course of the
disease and complications that may occurs due to disease or treatments
should be given to the parents. Explain that leukemia is not a contagious
disease, and patient still can interact with their friends or other family
members. At the initial phase of chemotherapy (induction phase for six
weeks), the patient will be given chemotherapy every week and should
be hospitalized for 2-3 days each week for administration of
intravenous and intrathecal chemotherapy. Oral chemotherapy drug
must be taken regularly at home. Compliance and supervision is
necessary, that chemotherapy can be given according to the protocol.
b. The side effects of chemotherapy
Side effects of chemotherapy depend mainly on the drugs the patient
receives. Hair loss, mouth sores, loss of appetite, diarrhea, nausea and
vomiting, increased risk of infections, bruising and bleeding easily, and
fatigue are some complications related to chemotherapy.
c. Prevention of infections
Patients with ALL and neutropenia are highly susceptible to infections.
Family participation is very important to prevent infections through
managing a healthy lifestyle, by always washing hands before contact
with the patient, after contact with the patient, after contact with body
fluids and environment around the patient. Washing hands thoroughly
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using six steps of hand washing. The patient should use a face mask and
maintain personal and family hygiene.
d. Vaccination
It is generally recommended that vaccines should not be given during
chemotherapy treatment. This is mainly due to vaccines need immune
system responses to work, and patients during chemotherapy treatment
may not achieve adequate responses.
e. Health insurance
The total length of therapy for most ALL treatment plans is 2 to 3 years.
Government health insurance is very helpful for costly medical
treatment and can protect family from financial hardship.
f. Long-term prognosis
Acute lymphoblastic leukemia is a malignancy with a poor prognosis.
The five years life expectancy of patients with ALL is about 80% and
high probability of progressive disease. It requires patient’s compliance
during treatment. The four years survival rate is about 60%. With
intensive chemotherapy, remission occur in approximately 98% of
patients, but 25-30% may experience disease recurrence.
g. Psychosocial problems and quality of life
Long-term problems of ALL patients are not only about the disease, but
also the impact of the disease on quality of life and daily activities at
home. Assessment of patient’s quality of life and the possibility of
psychosocial disorders are required with PedsQL inspection at least
every six months. If there is any irregularity, immediate intervention
must be done as early as possible.
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IX. Follow Up
Day Subjective Objective Aassessment Planning
(Date)
Day 10-11 - Pain in the Physical examination Acute lymphoblastic Therapy
(May 5-6, waist Present status leukemia (L2) high - Hydration 2000 mL/BSA with
2018) decreased. General condition : moderately ill risk (C91.0) D5%¼NS infusion plus 25 mEq
- Gum Consciousness : alert induction phase, sodium bicarbonate per 500 mL
bleeding Pulse rate : 90-98 beats/minute, regular hyperleukocytosis of D5%¼NS
(+) Respiratory rate : 20-24 times/minute, regular (D72.8), and well - Calorie requirements 2295
- No bone Temperature : 36.5-37.0oC nourished. kcal/day, protein requirements
pain Pain scale (FLACC):0 (no pain) 40.8 grams/day:
- No fever, 3 x meals (1500 kcal)
nausea, General status 2 x snacks (600 kcal)
vomiting Clot in gum, no active bleeding. Multiple enlarged (total calories 91.5% of RDA)
- Good daily lymph nodes in left and right pre-auricula, retro- - Dexamethasone tapering up dose
intake auricula, submandibular, cervical anterior and in one week from 0.5 mg/m2
- Normal posterior, supraclavicular, axilla, inguinal with BSA/day until 6 mg/m2 BSA/day
bowel diameters of 1-6 cm, consistency of solid, flat surface, per oral equivalent 6 mg every 8
movement not felt warm, soft and painless. The liver was palpable hours until June 16, 2018 than
and 2 cm below the xiphoid process and 2 cm below the tapering down until Jun 22, 2018.
urination costal arch, with sharp edges, soft consistency, smooth - Allopurinol 10 mg/kg/day equal
surface, and was painless. The spleen was palpable at to 200 mg every 8 hours orally
Schuffner V. - Sodium bicarbonate 1.5 tablets
oral if pH urine <6.5
Anthropometric status - PRC transfusion 500 ml
BW 51 kg, BH 166 cm, MUAC 24 cm, nutritional
- TC transfusion 4 kolf
status based on MUAC 90%
- Plan start chemotheraphy if
hemoglobin >8 g/dL and platelet
Laboratory results (May 5th, 2018)
>50.0 K/uL
leukocyte 185.30 K/uL (absolute neutrophil 13.17
Diagnosis
K/uL, absolute lymphosit 156.60 K/uL); hemoglobin
Evaluation of complete blood every
7.43 g/dL; hematocrit 23.18%; platelet 25.11 K/uL,
3 days; liver function, renal
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BUN 14 mg/dL, creatinine 0.89 mg/dL, natrium 128 function and electrolyte every 2
mmol/L, kalium 3.69 mmol/L, chloride 89.4 mmol/L, weeks.
calcium 8.5 mg/dL, Feritin 462.20 ng/mL, LDH 588 Echocardiography prior to
U/L administration of daunorubicin
Monitoring
Laboratory results (May 6th, 2018) Vital signs, side effects of
leukocyte 223.40 K/uL (absolute neutrophil 14.86 chemotherapy
K/uL, absolute lymphosit 191.00 K/uL); hemoglobin
6.96 g/dL; hematocrit 22.31%; platelet 26.56 K/uL
Urinalysis results: specific gravity 1.012; pH 8.0;
leukocytes 1/LFV, no erythrocytes, or crystals
Cumulative fluid balance : +60-100 ml

Urine production : 2.0 – 2.1 ml/kg/hour
Day 12-14 - Pain in the Physical examination Acute lymphoblastic Therapy
(May 7-9, waist Present status leukemia (L2) high - Hydration 2000 mL/BSA with
2018) decreased. General condition : moderately ill risk (C91.0) D5%¼NS infusion plus 25 mEq
- No gum Consciousness : alert induction phase, sodium bicarbonate per 500 mL
bleeding Pulse rate : 80-92 beats/minute, regular hyperleukocytosis of D5%¼NS
- No bone Respiratory rate : 18-22 times/minute, regular (D72.8), and well - Calorie requirements 2295
pain Temperature : 36.5-37.0oC nourished. kcal/day, protein requirements
- No fever, Pain scale (FLACC):0 (no pain) 40.8 grams/day:
nausea, 3 x meals (1500 kcal)
vomiting General status 2 x snacks (600 kcal)
- Good daily Multiple enlarged lymph nodes in left and right pre- (total calories 91.5% of RDA)
intake auricula, retro-auricula, submandibular, cervical - Dexamethasone tapering up dose
- Normal anterior and posterior, supraclavicular, axilla, inguinal in one week from 0.5 mg/m2
bowel with diameters of 1-6 cm, consistency of solid, flat BSA/day until 6 mg/m2 BSA/day
movement surface, not felt warm, soft and painless. The liver was per oral equivalent 6 mg every 8
and palpable 2 cm below the xiphoid process and 2 cm hours until June 16, 2018 than
urination below the costal arch, with sharp edges, soft tapering down until Jun 22, 2018.
consistency, smooth surface, and was painless. The - Allopurinol 10 mg/kg/day equal
spleen was palpable at Schuffner V. to 200 mg every 8 hours orally
18
- Sodium bicarbonate 1.5 tablets
Anthropometric status oral if pH urine <6.5
BW 51 kg, BH 166 cm, MUAC 24 cm, nutritional - TC transfusion 4 kolf
status based on MUAC 90% - Plan start chemotheraphy if
hemoglobin >8 g/dL and platelet
Cumulative fluid balance : +50-100 ml >50.0 K/uL
Urine production : 2.2 – 2.5 ml/kg/hour Diagnosis
Evaluation of complete blood every
3 days; liver function, renal
function and electrolyte every 2
weeks.
Plan echocardiography prior to
administration of daunorubicin
Monitoring
Vital signs, side effects of
chemotherapy
19
X. Prognosis
Ad vitam : dubius ad malam.
The patient was diagnosed with high risk ALL which
have a poor prognosis.
Ad functionam : dubia.
The patient has hyperleucocytosis, thrombocytopenia
and anemia that affect the organ function. Result of
quality of life assessment was low.
Ad sanactionam : dubius ad malam.
The remission rate of children with newly diagnosed
ALL after undergoing chemotherapy reaches 80%, but
20-25% may relapse.
19
XI. Scheme of Illness History
April 18th, 2018 April 26-30, 2018 May 1-4, 2018 May 5-9, 2018
End of March Day 6-9
Day 1- 5 Day 10-14
2018 Anamnesis
- Pain in the left and Anamnesis Anamnesis
Pain in the left and right waist Pain in the left and right Pain in the left and right waist,
Lumps in the neck, right waist, felt
Physical examination waist decrease decreased, gum bleeding (+)
armpits and in the more severe on the
Multiple enlarged lymph nodes in left and right Physical examination Physical examination
inguinal from 1 left waist.
pre-auricula, retro-auricula, submandibular, Multiple enlarged lymph Multiple enlarged lymph
month before - Abdominal nodes hepatosplenomegaly
enlargement cervical anterior and posterior, supraclavicular, nodes hepatosplenomegaly
admission. The size Laboratory results:
axilla, inguinal, diameters of 1-6 cm, consistency Laboratory results:
of the lump is about - Bone pain in both  CBC: hyperleucocytosis,
of solid, flat surface, not felt warm, soft and  CBC: hyperleucocytosis,
2-6 cm, initially on legs one week moderate anemia,
painless. hepatosplenomegaly moderate anemia,
the left neck, no before admission. trombocytopenia
Laboratory results: trombocytopenia
pain, no redness.  BMA: lymphoblast
 CBC: hyperleucocytosis, mild anemia,
General practitioners trombocytopenia infiltration 70%, with Assessment
varies size, suitable for Acute lymphoblastic leukemia
paracetamol and pain  Blood smear: immature cells with a low core
killer ALL L2. (L2) high risk induction
and cytoplasmic ratios, without granules 20%
Assessment phase, hyperleukocytosis, and
suspected acute leukemia (ALL differential
Acute lymphoblastic well nourished.
April 25th 2018 diagnosis AML). leukemia (L2) high risk Therapy
B general hospital Assessment induction phase, Hydration 3000 ml/BSA with
 referral to Sanglah Hyperleukocytosis, bicytopenia and hyperleukocytosis, and well IVFD D5 ¼ NS, allopurinol
Hospital with suspicion organomegaly et causa suspect acute leukemia
of hematological nourished. Indonesian Childhood ALL-
(ALL dd AML) differential diagnosis Therapy 2013 high risk protocol
malignancy
limphoma non-hodgin and well nourished Hydration 2000 ml/BSA chemotherapy
with IVFD D5 ¼ NS, PRC transfusion 500 ml
Therapy
allopurinol TC transfusion 8 kolf
Hydration 2000 ml/BSA with IVFD D5 ¼ NS,
allopurinol
Plan
BMA
20
XII. Scheme of Case Analysis
Infectious Agents Male, 15 years 2months Environmental exposure radiation exposure, chemicals exposure,
Risk Factors
alcohol, cigarettes, illicit drug use

Maternal reproductive history and birth Risk factor
Journal 1 Level of evidence 3a,
characteristics grade of recommendation B
(older parents age, very low or high birth)
weight) idiopathic
Fanconi anemia, Bloom syndrome,
Genetics
Down syndrome, Shwachman syndrome
Problems
Waist pain, bone pain, abdominal

enlargement, lymphadenopathy, ALL-L2 Long term Chemotherapy, imunization,
management nutrition, quality of life
hepatoslenomegaly, hyperleucocytosis
Risk of infection
Bone marrow aspiration Risk of malnutrition

Diagnosis
Anamnesis: Physical examination: Laboratory: hyperleukocytosis, moderate anemia, thrombocytopenia

Waist pain, lumps in neck, axilla, Lymphadenopathy, Bone marrow aspiration: ALL (L2)
inguinal, abdominal enlargement hepatosplenomegaly
Supportive therapy Emergency therapy Chemotherapy Complications therapy

Therapy
Hiperleukositosis
Pediatric Jurnal 2. level of evidence 2b, grades of Indonesian Childhood ALL-2013
nutritional care ~ recommendation B Protocol high risk
RDA
OPTIMAL
GROWTH AND
Ad vitam: dubius ad malam DEVELOPMENT
Prognosis
Survival and prognosis

Journal 3 level of evidence 2b, grade of Ad functionam: dubius
recommendation B Quality of life Ad sanactionam: dubius ad malam
Journal 4 level of evidence 1a, grade of Stimulating,
recommendation A parenting, caring
21
XIII. Case Analysis
Leukemia is classified into two major categories, acute and chronic leukemia. Acute
Leukemia is the major malignancy in childhood account for about 30-40%.1 Acute
leukemia represent a clonal expansion and arrest at a specific stage of normal
myeloid or lymphoid hematopoiesis. They constitute 97% of all childhood leukemia
and consist of the following types: acute lymphoblastic leukemia (ALL) about 75%;
acute myeloblastic leukemia (AML) about 20%; acute undifferentiated leukemia
(AUL) about 0.5%; and acute mixed-lineage leukemia (AMLL).2 Leukemia is a
malignancy derived from bone marrow, characterized by proliferation of white
blood cells, with the manifestation of abnormal cells in the peripheral blood.
Malignant cells replace normal cells due to a progressive expansion of malignant
cells group in bone marrow and then circulate systemically, and may be
accompanied by organ infiltration. This condition is more common in boys than
girls, and the peak incidence were found at age between 2-5 years.1,2 In this case,
the patient is a 15 years and 2 months old male with ALL (L2). Etiology of leukemia
in this patient is unknown but there was previous exposure to cigarettes smokes
from his father and brother.
Etiology of leukemia is unknown; some studies associate it with
environmental factors like ionizing radiation, chemical agents, and drugs. Other
factors are genetic factors, viral or bacterial infections, cigarettes exposure,
nonionizing radiation, or electromagnetic exposure.1,2 In this case, patient got the
father of the smokers (including when the mother is pregnant) and the patient
himself is often exposed to cigarette smoke. To find out the risk of exposure to
secondhand smoke on the incidence of leukemia in children, we conducted
evidence-based searches, and received the journal “Tobacco smoke and risk of
childhood acute lymphoblastic leukemia: findings from the SETIL case-control
study”, by Farioli et al., in Cancer Causes Control tahun 2014. This journal is valid,
important, and applicable (Level of evidence 3a, grades of recommendation B).
Journal conclusion is an increased risk of leukemia incidence in children of mothers
with exposure to secondhand smoke during pregnancy (passive smoking) and
children exposed directly to cigarette smoke. Fetal smoking in both conception and
pregnancy periods is not associated with an increased risk of leukemia in children.
22
This journal can be applied that at the time of giving of education to parents about
risk factor of leukemia in patient.
Signs and symptoms of ALL illustrate displacement of leukemia cells in
hematopoiesis system, lymphoid system, and extramedullary organ. Most frequent
systemic symptoms were fever (61%), bleeding (48%) and bone pain (23%), more
symptoms listed in table 1 below. Infiltration into the hematopoiesis system causes
anemia, neutropenia, and thrombocytopenia. Bleeding manifestation including
epistasis, hematoma, petechiae causes by thrombocytopenia. The signs of leukemia
infiltration to the lymphoid system manifest as lymphadenopathy, hepatomegaly,
and splenomegaly. Leukemia also can infiltrate to extramedullary organs, clinical
manifestations of this condition depend on organs involved; such as signs of
increased intracranial pressure in the central nervous system, testicular enlargement
in the genitourinary system, gastrointestinal tract bleeding in the gastrointestinal
tract, bone and joint pain, and others.2,3 From laboratory examination mostly
showed leukocytosis (53%), anemia (88%), and thrombocytopenia (75%).2
Table 1 Symptoms and clinical findings of patients with leukemia2
Symptoms and physical findings Percentage of Patients
Fever 61
Bleeding (e.g., petechiae or purpura) 48
Bone pain 23
Lymphadenopathy 50
Splenomegaly 63
Hepatosplenomegaly 68
In this case, we found hematopoiesis system, lymphoid system, and

extramedullary organ infiltration. The patient had complained gum bleeding due to
infiltration of leukemic cells to the bone marrow. Complete blood count showed
hyperleukocytosis, severe neutropenia and thrombocytopenia. Multiple
lymphadenopathy and hepatosplenomegaly were proving the lymphoid system
infiltration. Bone pain occurred due to extramedullary organ infiltration.
Diagnosis of ALL is made according to clinical and laboratory findings.
Complete blood count is a basic laboratory examination for patients suspected with
hematological malignancy. Bone marrow aspiration is performed to confirm the
23
diagnosis. Leukemia must be suspected when the bone marrow contains more than
5% blasts. The hallmark of acute leukemia diagnosis is the blast cell, a relatively
undifferentiated cell with diffusely distributed nuclear chromatin, one or more
nucleoli and basophilic cytoplasm.2 The latest recommendation from World Health
Organization (WHO), ALL should not diagnose based on bone marrow morphology
examination only, but also by immunophenotyping and cytogenetic examination.
Immunophenotyping examination can identify the strain (lineage) of leukemic cells
and the stage of maturation. Leukemia immunophenotyping is devided into three
groups based on the origin of the cancer cell precursors, including ALL pre-B cells,
ALL mature B cells, and ALL T cells. Most of ALL derived from B cell lines, while
only 10-15% comes from T cell lines. Acute lymphoblastic leukemia T cells are
more common in boys and more often accompanied by hyperleukocytosis,
mediastinum mass or enlargement of mediastinum lymph nodes.4-6 In this case, we
only performed bone marrow aspiration. From bone marrow aspiration (Picture 1.)
revealed hypercellularity with decreased on erythroid, myeloid, and megakaryocyte
system. Infiltration lymphoblast on bone marrow counted 70% with varies size,
From these findings, the patient was diagnosed acute lymphoblastic leukemia (L2).
A B
Picture 1. Bone marrow aspiration. (A) Fragments or particles of

marrow in bone marrow aspirate films, low power, Hypercellular particle.
(10x) (B)Limfoblast (100x)
24
For children with acute lymphoblastic leukemia, the identification of those at
higher risk of disease recurrence and modifying therapy based on this risk is a
critical component to the provision of optimal care. Determining risk in patients
with ALL is based on age, gender, and initial leukocyte count, involvement of the
central nervous system (CNS), testicular involvement, cytogenetic examination
results, and response to induction phase. High-risk patients are one or more of those
<1 year or >10 years of age, male, leukocyte count at baseline >50,000/µL,
involvement of central nervous system that is characterized by presence of blast
cells in cerebrospinal fluid (CSF) and leukocytes >5/µL or clinical evidence of CNS
involvement, painless enlarged testicle, hypodiploidy finding in cytogenetic
examination, t(9:22), 11q23, iAMP2, and lack of response after induction phase
therapy.2,7,8 In this case, patient are > 10 years old, male and complete blood count
evaluation showed leukocyte count >50,000/µL, then the patient was categorized
as high risk ALL.
Emergency oncology condition that can be found in children is
hyperleukocytosis, characterized by increased peripheral blood leukocyte counts
more than 100 x 103μL, whereas leukocyte counts more than 50 x 103μL referred
as leukocytosis. Hyperleukocytosis can be found in 6-15% of cases of acute
lymphocytic leukemia, 13-22% of all cases are non-acute lymphocytic leukemia
and nearly all cases are chronic myelogenous. This condition can induce metabolic
disorders due to lysis of leukemic cells. Metabolic disorders that known as tumor
lysis syndrome is consist of hyperuricemia, hyperkalemia, hyperphosphatemia and
secondary hypocalcemia, and sometimes lactic acidosis. Hyperleukocytosis can
also increase blood viscosity, an aggregation and blast cells thrombus in the
microcirculation. In addition, because blast cells are larger than mature leukocyte
cells, and not easily deformed, blast cells will be easily caught and cause occlusion
of the microcirculation. This condition is called leukostasis. Commonly, leukostasis
is appeared in central nervous system and lungs. Leukostasis will cause poor
perfusion and hypoxia, anaerobic metabolism, lactic acidosis, it will eventually
cause damage to blood vessel walls and induce bleeding.1,9
Management of hyperleukocytosis includes intensive supportive care and
cytoreduction. Hydration with intravenous fluid should be 2-3 times higher than
25
fluids requirement or 2-3 liters/BSA/day to get minimum diuresis of 2-3
ml/kg/hour. Hydration is best provided by potassium and calcium free fluids.
Administrations of 5% dextrose saline, 0.9% sodium chloride, or N/2 or N/4 5 %
dextrose were reported in some studies. Urine alkalinization can be achieved by
adding sodium bicarbonate to intravenous fluids as much as 40-60 mEq/L to
maintain urine pH between 7.0-7.5. Allopurinol at a dose of 10 mg/kg/day can
decrease plasma uric acid concentration.9,10 In this case, for clinical consideration,
hydration was given with D5¼NS 2000 ml/BSA + 25 mEq of sodium bicarbonate
per 500 ml D5¼NS and allopurinol 10 mg/kg/day equivalent to 200 mg every 8
hours (oral).
The prognosis of acute lymphocytic leukemia with hyperleukocytosis is
generally poor.1,5 In this case, hyperleukocytosis is obtained with leukocyte levels
between 145.90 to 209.80 x 103μL. We conducted a journal to find out the prognosis
of ALL patients who came first with hyperleucocytosis, obtained journal
“Childhood acute lymphoblastic leukemia with hyperleukocytosis at
presentation”, by Kong, et al., in journal Blood Research, 2014. The journal is
valid, important, and applicable (Level of Evidence 2b, grades of recommendation
B). The outpatients of ALL children with initial leukocyte counts >200 × 10 K/μL
are very poor, probably due to early toxicity leading to death during induction
therapy.
Management of ALL patient must be comprehensive, including supportive
and curative treatment. Treatment of ALL patients should include corresponding
diseases and treatment of complications such as provision of blood transfusion,
medication to increase granulocytes, anti-fungal drugs, good nutrition, and
management of psychosocial aspects. Curative treatment for ALL patients is
chemotherapy that includes induction, consolidation, intensification, and
maintenance phases. Chemotherapy is still the only treatment of children with ALL
although the cost is quite high. Some countries provide protocols to save costs while
still considering treatment outcomes. Standard treatment protocol is the Indonesian
Childhood ALL-2013 Protocol. In this protocol, patients are specified into two
groups based on severity of the disease, which are standard risk and high risk.
Suitable conditions for chemotherapy include hemoglobin levels ≥8-10 mg/dL,
26
leukocyte levels <50 K/µL, serum creatinine within normal limits and transaminase
enzyme levels did not exceed four times of upper normal limit.1,8 In this case, the
condition suitable for oral chemotherapy and he received chemotherapy treatment
based on Indonesian Childhood ALL-2013 High Risk Protocol.
Survival rates among teenagers with ALL are inferior to those among young
children, and survival is even worse among young adults. The reason for these
differences are multifactorial and include treatment factors, a higher prevalence of
unfavorable genetic subtypes among the older patients, the reduced ability of
teenagers and young adults to receive intensive therapy without untoward side
effects, and social factors such as insurance coverage and lack of parental
supervision of therapy.15 Although a rare disease, ALL presents a significant public
health burden given poor survival outcomes among adults, and its importance as
the most common pediatric cancer. Infection is the major cause of treatment-
related mortality (TRM) in childhood acute lymphoblastic leukemia.14 To
determine survival and prognostic factor for outcome of children with ALL we
performed journal searching and obtain evidence-based journal entitled “Improved
survival for children and adolescent with acute lymphoblastic leukemia
between 1990 and 2005: A report from the Childrens’s Oncology Group”, by
Hunger et al. 2012. This journal was valid, important, and applicable (level of
evidence 2b, grade of recommendation B). This journal concludes that 5 years
survival rate in children with ALL aged 1-9.99 years old was 90.4%, male patients
was 89.9%, while female patients was 91.0%. Patient with standard risk was 95.0%
while high risk was 82.9%. Risk of death higher in the group of children aged ≥15
years old and children with high risk. In this case, the patient is a 15 years and 2
months old male with high risk ALL. From this data and according to this journal
the overall survival for this patient range between 82.9 to 89,9%. This data is
important in giving information and education for the parent about the survival
chance of the patient.
According to the international literature, pediatric cancer patients in the acute
phase of the disease show reduced quality of life (QoL). Children with malignancy
are more likely to experience anxiety and depression that cause behavioral disorders
and low quality of life.9 To determine quality of life of children with ALL we
27
performed journal searching and obtain evidence-based journal entitled "Health-
related quality of life of children on treatment for acute lymphoblastic
leukemia: A systematic review" by Fardell et al. 2017. This journal was valid,
important, and applicable (level of evidence 1a, grade of recommendation A).
This journal concludes that quality of life values initially low at the beginning of
chemotherapy (induction phase) and will increase after maintenance phase of
chemotherapy, but it is highly depend on factors that worsen quality of life. Factors
contributing to a decline in the quality of life are a phase of intensification,
corticosteroid therapy, experiencing drug toxicity, greater age and female gender.
In this case, PedsQL evaluation results showed low quality of life. Based on
journal, patients receive a high-risk and long term protocol of chemotheraphy with
high toxicity, so the prognosis of this patient was ad functionam dubius ad malam.
XIV. References
1. Case analysis
1. Permono B, Ugrasena IDG. Leukemia akut. In: Permono B, Sutaryo,
Ugrasena IDG, Windiastuti E, Abdulsalam M, editors. Buku Ajar
Hematologi-Onkologi Anak. Jakarta: Badan Penerbit IDAI; 2010. p.
236-47.
2. Wexler LH, Wiener LS. Leukemias. In: Lanzkowsky P, editors.
Manual of Pediatric Hematology and Oncology. 5th ed. Amerika
Serikat: Elsevier; 2011. p. 518-66.
3. Smith OP, Hann IM. Clinical features and therapy of lymphoblastic
leukemia. In: Arceci RJ, Hann IM, Smith OP, editors. Pediatric
Hematology. 3rd ed. Australia: Blackwell Publishing; 2006. p. 450-81.
4. Peters JM, Ansari MQ. Multiparameter flow cytometry in the
diagnosis and management of acute leukemia. Arch Pathol Lab Med.
2011;135:44-54.
5. Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau M,
et al. The 2016 Revision to the World Health Organization
Classification of Myeloid Neoplasms and Acute Leukemia. Blood.
2016;127:2391-405.
28
6. Goldberg JM, Silverman LB, Levy DE, Dalton VK, Gelber RD,
Lehmann L, et al. Childhood T-cell acute lymphoblastic leukemia: the
Dana-Farber Cancer Institute acute lymphoblastc leukemia
consortium experience. J Clin Oncol. 2003;21:3616-22.
7. Bhojwani D, Howard SC, Pui CH. High-risk childhood acute
lymphoblastic leukemia. Clin Lymphoma Myeloma. 2009;9:1-18.
8. UKK Hematologi Onkologi IDAI. Panduan Protokol Pengobatan
Leukemia Limfoblastik Akut Anak-2013 (Indonesian Childhood
ALL-2013 Protocol). Jakarta: Ikatan Dokter Anak Indonesia; 2013.
p.9-24.
9. Kong GH, Seo JH, Jun SE, Lee BK, Lim YT. Childhood acute
lymphoblastic leukemia with hyperleukocytosis at presentation.
Blood Res. 2014;49:29-35.
10. Windiastuti E, Mulawi C. Gangguan metabolik pada leukemia
limfositik akut dengan hiperleukositosis. Sari Pediatri. 2002;4:31-35.
11. Mendes AV, Sapolnik R, Mendoca N. New guideline for the clinical
management of febrile neutropenia and sepsis in pediatric oncology
patients. J Pediatr. 2007;83:54-63.
12. Freifeld AG, Bow EJ, Septowitz KA, Boeckh MJ, Ito JL, Mullen CA,
et al. Clinical practice guidlines for the use of antimicrobal agents in
neutropenic patients with cancer: 2010 update by infectious diseases
society of America. Clin Inf Dis. 2011;52:56-93.
13. Saeidpoir M, Hamedi AK, Hanachi P. Pattern of bacterial and fungal
infection in neutropenic pediatric patients. Iran J Med Sci.
2008;33:202-8.
14. Owens JL, Hanson SJ, Mc Arthur JA, Mikhailov TA. The need for
evidence based nutritional guidelines for pediatric acute
lymphoblastic leukemia patients: acute and long term following
treatment. Nutrients.2013;5:4333-46.
15. Hunger AP, Mullighan C. Acute lymphoblastic leukemia in children.
N Engl J Med. 2015;373:1541-52.
29
2. Journal (evidence based practice)
1. Farioli A, Legittimo P, Mattioli S, Miligi L, Benvenuti A, Ranucci A,
dkk. Tobacco smoke and risk of childhool lymphoblastic leukemia:
Findings from the SETIL case-control study. Cancer Causes Control.
2014;25:683-9.
2. Kong SG, Seo JH, Jun SE, Lee BK, Lim YT. Childhood acute
lymphoblastic leukemia with hyperleukocytosis at presentation. Blood
Res. 2014;49:29-35.
3. Hunger SP, Devidas M, Camitta BM, Gaynon PS, Winick NJ, Raeman
GH, dkk. Improved survival for children and adolescent with acute
lymphoblastic leukemia between 1990 and 2005: A report from the
Children’s Oncology Group. J Clin Oncol. 2012:1-6.
4. Fardell JE, Vetsch J, Trahair T, Mateos MK, Grootenhuis MA, Touyz
LM, et al. Health-related quality of life of children on treatment for
acute lymphoblastic leukemia: A systematic review. Pediatr Blood
Cancer. 2017;1-13.
XV. Abbreviation
ALL : acute lymphoblastic leukemia
AML : acute myeloblastic leukemia
ANC : absolute neutrophil count
BCG : Bacillus Calmette-Guérin
BH : body height
BMA : bone marrow aspiration
BW : body weight
CBC : complete blood count
EBP : evidence based practice
Hb : hemoglobin
IBW : ideal body weight
IVFD : intravenous fluid drip
LFV : large field of view
LV : left ventricle
30
MCH : mean corpuscular hemoglobin
MCHC : mean corpuscular hemoglobin concentration
MCV : mean corpuscular volume
ml : milliliters
mg : milligrams
MUAC : mid-upper arm circumference
PDAM : perusahaan daerah air minum
KPSP : kuesioner pra skrining perkembangan
PedsQL : the pediatric quality of life inventory
PLT : platelet
PRC : packed red cells
QoL : quality of life
RDA : recommended dietary allowanced
RV : right ventricle
SD : standard deviation
TC : thrombocyte concentrate
WBC : white blood cell
31
ATTACHMENT
Attachement 1 Growth chart
IKB
1801772
7
Stature
for age
Ideal body
weight
Weight
for age
32
IKB
1801772
7
33
Attachement 2 Genetic Height Potential
Upper limit genetic

height potential
(176.5 cm)
Mid-parenteral
height
(168 cm)
Lower limit genetic

height potential
(159.5 cm)
34
Attachment 3. Pediatric symptom checklist-17 (PSC)-17
INTERNALIZING
No Internalizing Scale Never Sometimes Often
1 Feels sad, unhappy √
2 Seems to be having less fun √
3 Worries a lot √
4 Is down on him or herself √
5 Feels hopeless √
Internalizing score 1
EXTERNALIZING
No Externalizing Scale Never Sometimes Often
1 Fights with other children √
2 Does not listen to rules √
3 Does not understand other people’s √
feeling
4 Teases others √
5 Blames others for his or her troubles √
6 Refuses to share √
7 Takes things that do not belong to him √
or her
Externalizing score 0
ATTENTION
No Attention Scale Never Sometimes Often
1 Fidgety, unable to sit still √ `
2 Daydreams too much √
3 Distracted easily √
4 Acts as if driven by a motor √
5 Has trouble concentrating √
Attention score 0
Total Score: 1 (without behavioral disorders)
35
Attachment 4
PedsQL TM Cancer Module Version 3.0

TEEN REPORT (ages 13-18)
In the past ONE month, how much of a problem has has this been for you
PAIN AND HURT Never Almost Some- Often Almost

(problems with...) never times Always
1. I ache or hurt in my joints and/or
0 1 2 3 4
muscles
2. I hurt a lot 0 1 2 3 4
NAUSEA Never Almost Some- Often Almost

(problems with…) never times Always
1. I become seick to my stomach
0 1 2 3 4
when I have medical treatments
2. Food does not taste very good to
0 1 2 3 4
me
3. I become sick to my stomach when
0 1 2 3 4
I think about medical treatments
4. I feel sick to my stomach to eat
0 1 2 3 4
5. Some foods and smeels make me
0 1 2 3 4
sicks to my stomach
PROCEDURAL ANXIETY Never Almost Some- Often Almost

1. Needle sticks (i.e. injections, blood
0 1 2 3 4
tests, IV’s) hurts
2. I get scared when I have to have
0 1 2 3 4
blood tests
3. I get scared abaut having needle
sticks (i.e. injections, blood tests, 0 1 2 3 4
IV’s)
TREATMENT ANXIETY Never Almost Some- Often Almost

1. I get scared when I am waiting to
0 1 2 3 4
see the doctor
2. I get scared when I have to go to
0 1 2 3 4
the doctor
3. I get scared when I have to go to
0 1 2 3 4
hospital
36
WORRY Never Almost Some- Often Almost
1. I worry about side effects from
0 1 2 3 4
medical treatments
2. I worry about whether or not my
0 1 2 3 4
medical treatments are working
3. I worry that my cancer will come
0 1 2 3 4
back or relaps
COGNITIVE PROBLEMS Never Almost Some- Often Almost

1. It is hard for me to figure out what
0 1 2 3 4
to do when something bothers me
2. I have trouble solving math
0 1 2 3 4
problems
3. I have trouble writing school
0 1 2 3 4
papers or reports
4. It is hard for me to pay attention to
0 1 2 3 4
things
5. It is hard for me to remember what
0 1 2 3 4
I read
PERCEIVED PHYSICAL Never Almost Some- Often Almost

APPEARANCE never times Always
(problems with…)
1. I feel I am not good looking 0 1 2 3 4
2. I don’t like other people to see my
0 1 2 3 4
scars
3. I am embarrassed when others see
0 1 2 3 4
my body
COMMUNICATION Never Almost Some- Often Almost

1. It is hard for me to tell the doctors
0 1 2 3 4
and nurses how I feel
2. It is hard for me to ask the doctors
0 1 2 3 4
and nurses questions
3. Its is hard for me to explain my
0 1 2 3 4
illness to other people
Evaluation result : 75 (normal quality of life)
37
Attachment 5
PedsQL TM Cancer Module Version 3.0

PARENT REPORT for TEENS (ages 13-18)
In the past ONE month, how much of a problem has has this been for you
PAIN AND HURT Never Almost Some- Often Almost

(problems with...) never times Always
1. Aches in joints and/or muscles 0 1 2 3 4
2. Having a lot of pain 0 1 2 3 4
NAUSEA Never Almost Some- Often Almost

1. Becoming nauseates during medical
treatments 0 1 2 3 4
2. Food not tasting very good to

0 1 2 3 4
him/her
3. Becoming nauseated while
0 1 2 3 4
thingking about medical treatments
4. Feeling to nauseous to eat
0 1 2 3 4
5. Some foods and smells making
0 1 2 3 4
him/her nauseous
PROCEDURAL ANXIETY Never Almost Some- Often Almost

1. Needle sticks (i.e. injections,
blood tests, IV’s) causing him/her 0 1 2 3 4
pain
2. Getting anxious about having
0 1 2 3 4
blood drawn
3. Getting anxious abaut having
needle sticks (i.e. injections, blood 0 1 2 3 4
tests, IV’s)
TREATMENT ANXIETY Never Almost Some- Often Almost

1. Getting anxious when waiting to
0 1 2 3 4
see the doctor
2. Getting anxious about going to the
0 1 2 3 4
doctor
3. Getting anxious about going to the
0 1 2 3 4
hospital
38
WORRY Never Almost Some- Often Almost
1. Worrying about side effects from
0 1 2 3 4
medical treatments
2. Worrying about whether or not
his/her medical treatments are 0 1 2 3 4
working
3. Worrying that the cancer will come
0 1 2 3 4
back or relaps
COGNITIVE PROBLEMS Never Almost Some- Often Almost

1. Difficulty figuring out what to
do when something bothers 0 1 2 3 4
him/her
2. Trouble solving math problems 0 1 2 3 4
3. Trouble writing school papers or
0 1 2 3 4
reports
4. Difficulty paying attention to
0 1 2 3 4
things
5. Difficulty remembering what
0 1 2 3 4
he/she reads
PERCEIVED PHYSICAL Never Almost Some- Often Almost

APPEARANCE never times Always
(problems with…)
1. Feeling that he/she is not good
0 1 2 3 4
looking
2. Not liking other people to see
0 1 2 3 4
his/her scars
3. Being embarrassed about others
0 1 2 3 4
seeing his/her body
COMMUNICATION Never Almost Some- Often Almost

1. Difficulty telling the doctors
0 1 2 3 4
and nurses how he/she feel
2. Difficulty asking the doctors or
0 1 2 3 4
nurses questions
3. Difficulty explaining his/her illness
0 1 2 3 4
to other people
Evaluation result : 70 (normal quality of life)
39
EVIDENCE-BASED CRITICAL APPRAISAL
CASE
A 15 years 2 months old boy was B general hospital with suspicion of hematological
malignancy. Pain in the left and right waist complained about one week before
admission. The pain initially disappeared but was heavier from 3 days before
entering the hospital. Pain is felt more severe on the left waist. Abdominal
enlargement started a week before admission. Abdomen was getting bigger and
harder especially in the left upper abdomen. Patients occasionally felt that his
stomach is full, especially after eating. Patients also complained of lumps in the
neck, armpits and in the inguinal from 1 month before admission. The size of the
lump is about 2-6 cm, initially on the left neck, no pain, no redness. Bone pain in
both legs one week before admission. The pain cannot be described by the patient,
occurred especially at night and resolved without any medication. No swelling, or
previous trauma. On physical examination, multiple enlarged lymph nodes in left
and right pre-auricula, retro-auricula, submandibular, cervical anterior and
posterior, supraclavicular, axilla, inguinal with diameters of 1-6 cm, consistency of
solid, flat surface, not felt warm, soft and painless. Hepatosplenomegaly.
Nutritional status based on MUAC was 90% (well nourished). Laboratory findings
revealed severe hyperleukocytosis, mild-moderate anemia and thrombocytopenia.
Blood smear: immature cells with a low core and cytoplasmic ratios, without
granules 20% suspected acute leukemia (ALL differential diagnosis AML). Result
of bone marrow aspiration revealed hypercellular, decreased erythroid system,
decreased myeloid system, decreased megacaryocyte system, lymphoblast
infiltration 70%, with varies size, suitable for ALL L2.
DIAGNOSIS
Acute lymphoblastic leukemia (L2) high risk (C91.0), hyperleukocytosis (D72.8),
and well nourished.
40
PROBLEMS
1. Can exposure of cigarette smoke be a risk factor for ALL compared to
without exposure of cigarette smoke?
2. What is complications and outcomes of pediatric patients with
hyperleukocytic acute lymphoblastic leukemia during induction therapy?
3. How is prognostic and what factors affect the prognosis and survival among
children with acute lymphoblastic leukemia?
4. How is the quality of life of children with acute lymphoblastic leukemia?
41
PROBLEM 1
PICO
According to the problems, PICO can be described as follows:
P (Patient/Problem) : children
I (Intervention) : active cigarette smokers exposure
C (Comparation/Control) : non active cigarette smokers exposure
O (Outcome) : risk of ALL
CLINICAL QUESTION
In children with ALL, does exposure to active cigarettes smokers from parents be
a risk factor for ALL than without exposure to active cigarettes smokers from
parents?
JOURNAL SEARCHING STRATEGY
Keywords: acute lymphoblastic leukemia AND parental smoking
RESULT
" Tobacco smoke and risk of childhood lymphoblastic leukemia: Findings
from the SETIL case-control study."
Farioli A, Legittimo P, Mattioli S, Miligi L, Benvenuti A, Ranucci A, dkk.
Cancer Causes Control. 2014; 25:683-9.
ABSTRACT
Background: Tobacco smoke could cause childhood acute lymphoblastic leukemia
(ALL) through at least three pathways: (1) prenatal parental smoking; (2) fetal
exposure through maternal smoking during pregnancy; and (3) childhood exposure
to secondhand smoke (SHS). We tested these hypotheses in a large population-
based case–control study (SETIL) primarily designed to evaluate the role of
electromagnetic fields in childhood hematopoietic malignancies.
Methods: from 1998 to 2003, we enrolled 602 incident cases of ALL from 14
Italian Regions, and 918 controls were individually matched by birthdate, sex, and
area of residence. Cases (n = 557) and controls (n = 855) with complete information
were analyzed; odds ratios (OR) and 95 % confidence intervals (95 % CI) were
estimated with logistic regression models conditioned on matching variables and
42
adjusted by birth order, birthweight, duration of breastfeeding, parental age at
delivery, education, and occupational exposure to benzene.
Results: No evidence associating paternal smoking in the conception period or
maternal smoking during the pregnancy with ALL was found. An association of
ALL with maternal exposure to SHS during pregnancy (adjusted OR for mothers
exposed more than 4 h/day = 2.18, 95 % CI 1.39–3.42) was observed, but recall
bias cannot be exclu- ded. Exposure of the children to SHS was associated with
ALL only in unadjusted analysis (unadjusted OR for highly exposed children =
1.64; 95 % CI 1.10–2.45).
Conclusions: This study does not support the hypothesis that parental active
smoking is associated with ALL. We found very weak evidence of increased risk
of ALL for children exposed to SHS. Maternal exposure to SHS was associated
with ALL, but recall bias is likely to inflate our estimates.

(CAUSATION ASPECT)
Is the study design valid?
1. Were there clearly defined groups of Yes
patients, similar in all important ways
other than exposure to the treatment
or other causes?
2. Were treatments/exposures and Yes
clinical outcomes measured in the
same way for both groups? Was the
assessment of outcomes either
objective or blinded to exposure?
3. Was the follow-up of study patients Yes
sufficiently long for the outcome to
occur?
4. Is it clear that the exposure preceded Yes
the onset of the outcome?
5. Is there a dose-response gradient? Yes
6. Is the association consistent from Yes
study to study?
7. Does the association make biological Yes
sense?
This study is valid.
43
Are the results important?
1. How strong is the Parents smoke during OR 95% CI
association between the period of conception
exposure and outcome - Never smoked 1.00 Ref
(seen from 95% IK - ex-smokers 0.67 0.47 – 0.95
value)? - 1-10 cigarettes / day 0.86 0.58 – 1.26
-> 11 cigarettes / day 0.74 0,51 – 1.05
P trend 0.33
Mother smokes in the

1st trimester
- Never smoked 1.00 Ref
- ex-smokers 1.05 0.79 – 1.40
- 1-10 cigarettes / day 1.05 0.66 – 1.65
-> 11 cigarettes / day 1.37 0.53 – 3.51
P trend 0.68
Exposure of SHS to the

mother during
pregnancy 1.00 Ref
- not exposed 1.26 0.83 – 1.90
1.99 1.26 – 3.15
- <2 hours / day
2.18 1.39 – 3.42
- 2-4 hours / day
-> 4 hours / day
P trend <0.001
Cumulative SHS
exposure in children
- not exposed
- <1000 cigarettes 1.00 Ref
- 1000-6999 cigarettes 0.89 0.57 – 1.40
-> 7000 cigarettes 0.89 0.52 – 1.53
P trend 0.95 1.03 0.63 – 1.69
This study is important.
Are the results important for my patient?

1. Is my patient so different from those No. Characteristic type of subjects in
included in the study that its results this study similar with our patients.
don’t apply?
2. What is my patient’s risk of the Yes, this evidence is worth for our
adverse event? What is my patient’s clinical practices.
potential benefit from therapy?
3. What are my patient’s preferences, By knowing the relationship between
concerns and expectations from this cigarette exposure with the
treatment? occurrence of ALL it is expected to
help reduce the incidence of ALL by
44
avoiding exposure to cigarettes in the
mother during pregnancy.
This study is applicable.
Conclusion: valid, important, and applicable.
Level of evidence 3a with grade of recommendation B.
45
PROBLEM 2
PICO
P (Patient/Problem) : Children with acute lymphoblastic leukemia
I (Intervention) :-
C (Comparation/Control) : hyperleucocytosis
O (Outcome) : Survival
CLINICAL QUESTION
In children with acute lymphoblastic leukemia, how is the survival of children
accompanied by hyperleucocytosis?
Keywords: Children with acute lymphoblastic leukemia AND hyperleukocytosis
AND prognosis
RESULT
“Childhood acute lymphoblastic leukemia with hyperleukocytosis at
presentation.”
Kong SG, Seo JH, Jun SE, Lee BK, Lim YT.
Blood Res. 2014;49:29-35.
ABSTRACT
Background: Hyperleukocytosis caused by acute lymphoblastic leukemia (ALL)
is associated with early morbidity and mortality due to hyperviscosity arising from
the excessive number of leukocytes.This study was designed to assess the incidence
of hyperleukocytosis, survival outcomes, and adverse features among pediatric
ALL patients with hyperleukocytosis.
Methods: Between January 2001 and December 2010, 104 children with
previously untreated ALL were enrolled at the Pusan National University Hospital.
All of them were initially stratified based on the National Cancer Institute (NCI)
risk; 48 (46.2%) were diagnosed with high-risk ALL. The medical charts of these
patients were retrospectively reviewed.
Result: Twenty (19.2%) of the 104 children with ALL had initial leukocyte counts
of >100×109/L, and 11 patients had a leukocyte count of >200×109/L. Male gender,
T-cell phenotype, and massive splenomegaly were positively associated with
46
hyperleukocytosis. Common early complications during induction therapy included
renal dysfunction, and central nervous system hemorrhage. The complete remission
(CR) rate for the pediatric ALL patients with hyperleukocytosis (94.1%) was
similar to the overall CR rate (95.6%). The estimated 3-year event free survival
(EFS) and overall survival of ALL children with hyperleukocytosis were 75.0%
and 81.2%, respectively. However, patients with initial leukocyte counts
>200×109/L had a lower EFS than those with initial leukocyte counts 100‒
200×109/L (63.6% vs. 100%; P=0.046).
Conclusion: The outcome of pediatric ALL cases with an initial leukocyte count
>200×10 /L was very poor, probably due to early toxicity-related death during
induction therapy.
(PROGNOSTIC ASPECT)
Are the results of this prognostic study valid?
1. Was a defined, representative Yes. The researcher clearly identifies
sample of patients assembled at a the subject of the study (page 30).
common (usually early) point in the
course of their disease?
2. Was patient follow-up sufficiently Yes. Data were taken from medical
long and complete? records at Pusan National University
Hospital from January 2001-
December 2010. There is no loss to
follow up in this study, so it can be
concluded a complete research.
3. Are clinical outcomes and clinical Yes. The researcher defines objective
outcomes measured in the same (life expectancy) objectively (page
way in both groups? 32)
4. If subgroups with different Yes
prognoses are identified, was there
adjustment for important prognostic
factors?
5. Was there validation in an Not explained in the study.
independent group (“test set”) of
patients?
47
Are the valid results of this prognostic study important?
1. How likely are the outcomes over During the monitoring, mortality in
time? cancer patients with
hyperleukocytosis at diagnosis was
obtained
Leukocytes <100 x 10K/μL of 21.4%
Leucocytes> 200 x 10K/μL of 27.2%
(Table 4 page 32).
2. How precise are the prognostic The association of
estimates? hyperleucocytosis with early
complications and the association
of hyperleukocytosis with
therapeutic response can be seen
in Table 3 and Table 4, on page 32.
Can we apply this valid, important evidence about prognosis in caring for our
patient?
1. Were the study patients similar to Yes, characteristic type of subjects in
our own? this study similar with our patients
2. Will this evidence make a clinically Yes, this evidence is worth for our
important impact on our conclusions clinical practices.
about what to offer or tell our
patient?
Level of evidence 2b with grade of recommendation B.
48
PROBLEM 3
PICO
I (Intervention) :-
C (Comparation/Control) :-
O (Outcome) : Prognosis
CLINICAL QUESTION
How is prognostic and what factors affect the prognosis and survival among
children with acute lymphoblastic leukemia in Indonesia?
Keywords: Children with acute lymphoblastic leukemia AND prognosis AND
survival
RESULT
“Improved survival for children and adolescent with acute lymphoblastic
leukemia between 1990 and 2005: A report from the Children’s Oncology
Group”
Hunger SP, Devidas M, Camitta BM, Gaynon PS, Winick NJ, Raeman GH, et al.
J Clin Oncol. 2012:1-6.
ABSTRACT
Background: To examine population-based improvements in survival and the
impact of clinical covariates on outcome among children and adolescents with acute
lymphoblastic leukemia (ALL) enrolled onto Children’s Oncology Group (COG)
clinical trial between 1990 and 2015.
Methods: in total, 21.626 persons age 0 to 22 years were enrolled onto COG ALL
clinical trials from 1990 to 2005, representing 55.8% of ALL cases estimated to
occur among US persons younger than age 20 years during this period. This period
was divided into three eras (1990-1994, 1995-1999, and 2000-2005) that included
similar patient numbers to examine changes in 5- and 10- year survival over time
49
and the relationship of those changes in survival to clinical covariates, with
additional analyses of cause of death.
Results: five-year survival rates increased from 83.7% in 1990-2005 (P<.001).
survival improved significantly in all subgroups (except for infants age≤1 year),
including males and females; those age 1 to 9 years, 10+ years, or 15+ years; in
whites, blacks, and other races; in Hispanics, non-Hispanics, and patients of
unknown ethnicity; in those with B-cell or T-cell immunophenotype; and in those
with National Cancer Institute (NCI) standard- or high-risk clinical features.
Survival rates for infants changed little, but death following relapse/disease
progression decreased and death related toxicity increased.
Conclusions: this study documents ongoing survival improvements for children
and adolescents with ALL. Thirty-six percent of deaths occurred among children
with NCI standard-risk features emphasizing that efforts to further improve survival
must be directed at both high-risk subsets and at those children predicted to have
an excellent chance for cure.

(PROGNOSTIC ASPECT)
Are the results of this prognostic study valid?
1. Was a defined, representative Yes, patients were recruited after
sample of patients assembled at a diagnosis of acute lymphoblastic
common (usually early) point in the leukemia.
course of their disease?
2. Was patient follow-up sufficiently Yes, median observations were 15
long and complete? years.
3. Are clinical outcomes and clinical Yes, the outcome of this is survival
outcomes measured in the same rate and prognostic factors.
way in both groups?
4. If subgroups with different Yes, explained in table 3.
prognoses are identified, was there
adjustment for important prognostic
factors?
5. Was there validation in an Not explained in the study.
independent group (“test set”) of
patients?
50
Are the valid results of this prognostic study important?
1. How likely are the
outcomes over
time?
2. How precise are
the prognostic
estimates?
Can we apply this valid, important evidence about prognosis in caring for our
patient?
1. Were the study patients similar to Yes, characteristic type of subjects in
our own? this study similar with our patients.
2. Will this evidence make a clinically Yes, this evidence is worth for our
important impact on our conclusions clinical practices.
about what to offer or tell our
patient?
Level of evidence 2b with grade of recommendation B.
51
PROBLEM 4
PICO
I (Intervention) :-
C (Comparation/Control) :-
O (Outcome) : quality of life
CLINICAL QUESTION
How is the quality of life of children with acute lymphoblastic leukemia?
Keywords: Children with acute lymphoblastic leukemia AND quality of life
RESULT
"Health-related quality of life of children on treatment for acute lymphoblastic
leukemia: A systematic review"
Fardell JE, Vetsch J, Trahair T, Mateos MK, Grootenhuis MA, Touyz.LM,
Marshall GM, Wakefield CE.
Pediatr Blood Cancer. 2017:1-13.
ABSTRACT
Background: This review aims to evaluate health related quality of life (HRQL) in
comparison to other groups (e.g., healthy controls, other diagnoses), to synthesize
the literature describing HRQL over the course of ALL treatment, and to identify
correlates of HRQL in children on treatment for ALL.
Methods: we conducted a systematic literature review on HRQL of ALL patients
on treatment according to PRISMA guidelines. We screened titles and abstracts of
all papers and included them for full text review if they contained information on
HRQL in ALL patients on treatment.
Results: all studies reported lower HRQL outcomes for ALL patients when
compared with healthy norms or siblings. When compared to AML patients, ALL
patients had worse scores. Quality of life values initially low at the beginning of
chemotherapy (induction phase) and will increase after maintenance phase of
52
chemotherapy, but it is highly depend on factors that worsen quality of life. Factors
contributing to a decline in the quality of life are a phase of intensification,
corticosteroid therapy, experiencing drug toxicity, greater age and female gender.
Conclusions: Understanding HRQL across ALL treatment allows at risk patients
to be identiﬁed early and offered intervention or support with the potential to alter
HRQL while on treatment and beyond.

(SYSTEMATIC REVIEW PROGNOSTIC ASPECT)
Is the systematic review valid?
1. Is this a systematic review or Yes.
meta-analysis that is expected Twenty-two studies, including 2073 patients,
to answer our clinical fulfilled inclusion criteria which data
questions? collection and analysis were performed
according to PRISMA guidelines.
2. Is the methodology mentioned? Yes.
How to get relevant articles? This included searches in PubMed (including
Medline), PsyInfo, Embase, and the
Cochrane database without language
restrictions and include all years up to March
2, 2016
How to assess the validity of Two reviewers assessed the quality of the
each article? studies, after assessing the first 20% of
studies quality ratings together. Interrater
reliability was over 90%, disagreements were
resolved by discussion with third reveiwer.
3. Is there a possibility that No
important research results are All clinical trials which met requirements for
not included in this meta- this systematic review already included.
analysis?
4. Are the results of each study Yes
consistent? The results of twenty two studies included in
this journal has similar results.
53
Is this valid systematic review is important?
1. Is the overall outcome clinically Yes.
important so that we will apply to The outcome is clinically important.
our patients?
2. If there is an analysis of Yes.
subgroups, do we believe the
subgroup results are important?
3. Are the clinically significant Yes.
results statistically significant? Most studies reported that all domains of
HRQL were affected by ALL treatment. The
physical, psychosocial, social, emotional, and
school domains were most commonly
measured, and all were reported to be lower
in ALL patients than in healthy children and
siblings.
Can the valid and important systematic review be applied to our patients?
1. Are the characteristics of our Yes
patients similar to those present in The characteristics of our patients is similar.
this meta-analysis?
2. Are medications, expertise, Yes
facilities available, required? Medications, expertise, and facilities are
available
3. Can the patient and family receive Yes
medication / treatment based on Therapy given was not contradicted with our
social, cultural, religious values? social values and religious culture.
Level of evidence 1a with grade of recommendation A.
54

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EVIDENCE BASED CASE

ACUTE LYMPHOBLASTIC LEUKEMIA (L2) HIGH

POSTGRADUATED PEDIATRIC TRAINING PROGRAM

II. History Taking (Subjective)

III. History During Admission Until Determination as Case

IV. Physical Examination (Objective) on May 4, 2018

Neurological examination of four limbs:

d. Anthropometric status (based on WHO growth chart 2006)

6. Laboratory and radiology findings

Cumulative fluid balance : +60-100 ml

alcohol, cigarettes, illicit drug use

Waist pain, bone pain, abdominal

Bone marrow aspiration Risk of malnutrition

Anamnesis: Physical examination: Laboratory: hyperleukocytosis, moderate anemia, thrombocytopenia

Supportive therapy Emergency therapy Chemotherapy Complications therapy

Survival and prognosis

In this case, we found hematopoiesis system, lymphoid system, and

Picture 1. Bone marrow aspiration. (A) Fragments or particles of

Upper limit genetic

Lower limit genetic

Total Score: 1 (without behavioral disorders)

PedsQL TM Cancer Module Version 3.0

PAIN AND HURT Never Almost Some- Often Almost

NAUSEA Never Almost Some- Often Almost

PROCEDURAL ANXIETY Never Almost Some- Often Almost

TREATMENT ANXIETY Never Almost Some- Often Almost

COGNITIVE PROBLEMS Never Almost Some- Often Almost

PERCEIVED PHYSICAL Never Almost Some- Often Almost

COMMUNICATION Never Almost Some- Often Almost

PedsQL TM Cancer Module Version 3.0

PAIN AND HURT Never Almost Some- Often Almost

NAUSEA Never Almost Some- Often Almost

2. Food not tasting very good to

PROCEDURAL ANXIETY Never Almost Some- Often Almost

TREATMENT ANXIETY Never Almost Some- Often Almost

COGNITIVE PROBLEMS Never Almost Some- Often Almost

PERCEIVED PHYSICAL Never Almost Some- Often Almost

COMMUNICATION Never Almost Some- Often Almost

EVIDENCE-BASED CRITICAL APPRAISAL

Mother smokes in the

Exposure of SHS to the

Are the results important for my patient?

This study is important.

EVIDENCE-BASED CRITICAL APPRAISAL

This study is important.

EVIDENCE-BASED CRITICAL APPRAISAL

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