HEMATOLOGIC DISORDERS

Care of Clients with Hematologic Diseases

• Blood – transports cellular requirements and

products from one part of the body to another

• composed of plasma (55%) and cellular

component (45%)

– 5-6 liters or 70-75 ml/kg BW (average

volume)
 Care of Clients with Hematologic Diseases

• Hematopoiesis – blood cell production; done in the bone

marrow (red), pelvis, sternum, ribs, epiphysis of long
bones
• Erythropoiesis – red blood cell production in the liver in
utero (2-5months) then in bone marrow.
– needs iron, protein, pyridoxine (B6), cyanocobalamine (B12),
folic acid, and copper
• Reticuloendothilial System – mononuclear phagocyte
system or macrophage (spleen, liver, lymphatic system,
lungs)
 Care of Clients with Hematologic Diseases

• Diagnostic Assessment:
• Blood
– CBC with differential
a. Hemoglobin – Males13-16 gm/dl
- Females 12-14 gm/dl
b. Hematocrit – Males 42-50%
- Females 40-48%
c. RBC – N=Males 4,600,000 – 6,200,000 per cu.mm
Females 4,200,000 – 5,400,000 per cu.mm
 Diagnostic Assessment

• Blood
d. WBC – N=5,000 – 10,000 cu.mm
*neutrophils – N=60-70%
*eosinophils – N=1-4%
*basophils – N=0–0.5%
*monocytes – N=2-6%
*lymphocytes – N=20-30%
e. Platelets – N=200,000-350,000 per cu.mm
 Diagnostic Assessment

Miscellaneous
a. ESR – N=0 to 20 mm/hr
b. Coomb’s test – indirect blood from mom, direct blood
from baby’s cord
c. Schillings test – Vit.B12 in the gastro-intestinal system
prep NPO x 8 hours
radioactive Vit.B12 given PO
Vit.B12 nonradioactive given IM
2 hours after urine collection for radioactive
Vit.B12; N = 15-40% of oral dose excreted
 Diagnostic Assessment
• Urine and Stool
– Urinalysis
– Hematest
– Hemoccult – prep; -no dark colored food x 24 hours prior to
test
• Radiologic
– CXR
– Lymphangiography
• Bone Marrow aspiration and biopsy
– Preferred site – iliac crest, sternum or tibia
– Before: consent, position exposing the site
– After: pressure to site x5minutes
 Care of Clients with Hematologic Diseases

• Nursing Assessment:
– Pallor – conjunctiva
– Jaundice (hemolytic) – sclera; palms of hands;
soles of feet
– Signs of bleeding such as petechiae, ecchymosis,
hematoma, epistaxis
– Lymph nodes enlargement
– Limited joint range of motion
– Splenomegaly or hepatomegaly
ANEMIA
 ANEMIA
• A condition in which the hemoglobin
concentration is lower than normal,
reflects fewer than normal RBCs within
the circulation

• Not a disease in itself but a sign of an

underlying disorder
 Classification of Anemia
accdg to etiology:
1. Hypoproliferative Anemias
- from decreased RBC production caused by a
deficiency in cofactors required for erythropoiesis,
bone marrow is suppressed or is inadequately
stimulated because of a lack of erythropoietin

2. Bleeding
– from RBC loss (500ml or <)

3. Hemolytic
- from increased destruction of RBCs which may
occur because of an overactive RES or the bone
marrow produces abnormal RBCs that are then
destroyed by the RES
 ANEMIA: Clinical Manifestations

Tissue hypoxia - underlying cause of

all manifestations accompanying
anemia
• Common:
– pallor - palpitations
– easy fatigability - hypotension
– SOB - anorexia
– dyspnea on exertion
– tarry stools
– weight loss - headache
– weakness - dizziness
IRON DEFICIENCY
ANEMIA
 Iron Deficiency Anemia
• Signs and Symptoms:
– Palpitations, dizziness, easy fatigability
– Cold sensitivity, pallor
– Brittle nails, and hair
– Plummer-vinsons syndrome – soreness and
inflammation of mouth and tongue (stomatitis
and glossitis)
• IDA
– Poor iron intake
– Most common during
• 6mos-2 yrs
• Adolescents
– May also be caused by chronic disease

– DX
• CBC
 Iron Deficiency Anemia
• Nursing management:
*Oral iron – route of choice; given after meals
- liquid iron intake with straw because it stains
- best absorbed with Vitamin C
- stool becomes tarry and constipation may occur.
*Parenteral – avoid tissue staining by using separate
aspiration injection needles
- Z-track method and deep IM

*Dietary – increased in iron and roughage

*Blood transfusion
 IDA: Nursing Management
• Preventive Education- especially in menstruating and
pregnant women

• Diet: Iron-rich foods such as organ meats, beans,

leafy green vegetables, raisins

• Promote rest to reduce oxygen demands.

MEGALOBLASTIC
ANEMIAS
 MEGALOBLASTIC ANEMIAS
• anemias caused by deficiencies of Vitamin B12
and folic acid
• characterized by appearance of megaloblasts
(abnormally large, primitive RBCs) in blood and
bone marrow
 MEGALOBLASTIC ANEMIAS
• Folic Acid Deficiency
Folic acid - a vitamin necessary for normal RBC
production, stored as folates

Deficiency - occurs in people who rarely eat uncooked

vegetables

Increased folic acid requirements - alcoholism, pt

with chronic hemolytic anemias, pregnant women
(spina bifida)

Management:
1. Eat source
2. Take folic acid
 MEGALOBLASTIC ANEMIAS
• Vitamin B12 Deficiency
– inadequate intake, can develop in strict
vegetarians who consume no meat or dairy
products
– intrinsic factor - normally secreted by cells
within the gastric mucosa, and binds with
dietary Vit. B12 and travels with it to the
ileum
– PUD, gastric resections, ileum problems

– PERNICIOUS ANEMIA
 MEGALOBLASTIC ANEMIAS
Clinical Manifestations
• Typical manifestations of anemia (weakness,
listlessness, fatigue)
• Vit. B12 deficiency - neurologic manifestations

• Pernicious anemia: smooth, sore, red tongue

and mild diarrhea, extremely pale, paresthesia
difficulty maintaining their balance because of
damage to the spinal cord and also lose position
sense
Management of Vit B12 deficiency
• Increase dietary intake of vitamin B12 (animal
proteins, eggs, dairy products)
• Vitamin B12 injections weekly at first then
monthly for maintenance
APLASTIC ANEMIA
 APLASTIC ANEMIA
• Decrease in or damage to marrow stem cells, damage to
the microenvironment within the marrow, and
replacement of the marrow with fat

• results in bone marrow aplasia (markedly reduced

hematopoiesis) which results in anemia, leukopenia and
thrombocytopenia
 APLASTIC ANEMIA Clinical
Manifestations
• Pancytopenia
- normocytic anemia
- neutropenia
- thrombocytopenia
 APLASTIC ANEMIA Medical
Management
• Bone marrow
transplantation
• Peripheral blood stem
cell transplantation
• Immunosuppressive
therapy
 APLASTIC ANEMIA Nursing
Management
• Assess for signs of:
* Infection- Eliminate intake of raw fruits, vegetables
and raw meat , practice asepsis; reverse/ protective
isolation, limit visitors
*Bleeding- Avoid ASA administration; use of soft
bristled toothbrush, avoid enemas, avoid picking nose,
avoid trauma, use electric razor
* Fatigue- rest, avoid strenuous activity
 LEUKEMIA
• Classification:
– According to stem cell line involved:
• Lymphoid
• Myeloid
– According to onset:
• acute
• chronic
 Leukocyte Disorders
• Leukemia – most common of childhood (3-5
y/o) cancer; abnormal proliferation of WBC in
blast form.
Predisposing factors:
– Radiation
– Survivors of Hiroshima
– Benzol, aniline dyes
 Leukemia
• Types of Leukemia:
*Acute lymphocytic leukemia (ALL)
• 80-85%of childhood leukemia
• 95% chance of obtaining remission with diagnostic
assessment
• 75% chance of surviving over 5 years
*Acute non-lymphocytic anemia (ANLL)
• granulocytic and monocytic
• 60-80% will obtain remission with treatment
• 30-40% cure rate
ACUTE MYELOID LEUKEMIA (AML)
• results from a defect in the hematopoietic
stem cell that differentiates into all myeloid
cells
• incidence rises with age (peak at age 60
years)
• prognosis is highly variable
• 1-3 years with chemotherapy; 2-5 months
Without chemotherapy
ACUTE MYELOID LEUKEMIA (AML)

• CLINICAL MANIFESTATIONS
– fever & infection (neutropenia)
– weakness & fatigue (anemia)
– bleeding tendencies (thrombocytopenia)
– pain from an enlarged liver or spleen
– hyperplasia of gums
– bone pain
ACUTE MYELOID LEUKEMIA (AML)

• DIAGNOSTIC FINDINGS
– CBC
• decrease mature leukocytes, erythrocytes &
platelets
• total leukocyte count can be low, normal, or
high
– Bone Marrow Analysis
• increase of immature blast cells (more than
30%)
ACUTE MYELOID LEUKEMIA (AML)
• MEDICAL MANAGEMENT
– Bone Marrow Transplantation (BMT)
• infusion of donor stem cell to initiate blood stem cell
production
• Types:
– Allogenic BMT
– Umbilical Cord Blood Stem Cell Transplantation
– Autologous BMT
– Peripheral Blood Stem Cell Transplantation- a type of
autologous transplant
 CHRONIC MYELOID LEUKEMIA
• arises from mutation in myeloid stem cell
• normal myeloid cells continue to be
produced
• preference for immature forms
• uncommon in people <20 yrs old
• incidence increases with age
 THROMBOCYTOPENIA

• blood disease
characterized by
an abnormally
small number of
platelets in the
blood Normal = 150,000 – 400,000
< 20,000 /mm3 (Serious )
<10,000/mm3 (Potentially life
threatening )
 Causes:
A. Decreased production of platelets
within the bone marrow

B. Increased consumption of platelets

– Disseminated intravascular coagulation
 Causes:
C. Increased destruction of platelets
- in the bloodstream (intravascular)
• Due to antibodies
– ITP, Lupus erythematosus, Malignant lymphoma
• Due to infection
– Bacteremia, post-viral infection
– in the spleen or liver (extravascular)
• Hypersplenism /enlarged spleen - Sequestration
of platelets in spleen
 Clinical Manifestations
• Platelet counts > 50,000/mm3
– Bleeding and petechiae usually do not occur
– Excessive bleeding can follow surgery

• Platelet count < 20,000/ mm3

– Petechiae
– Nose and gingival bleeding
– Excessive menstrual bleeding
– Excessive bleeding after surgery or dental extractions

Platelet count < 5,000/ mm3

– Spontaneous, potentially fatal central nervous system or
gastrointestinal hemmorrhage
 Medical Mgt:
• Treatment of underlying disease
• Platelet transfusions
• FFP
• Splenectomy
 Pharmacological Mgt
• Vitamin K
• Prednisone (immunosuppressive agent)
• Gamma Globulin (IVIg)
• Rh0 [D] Immune Globulin (WinRho)
 Disseminated Intravascular
Coagulation
• Not a disease
• May be triggered by sepsis, trauma,
cancer (especially prostate cancer and
acute promyelocytic leukemia), shock,
abruptio placentae,toxins or allergic
reactions
• Potentially life-threatening
 Pathophysiology
• Normal hemostatic mechanisms are altered so
that a massive amount of tiny clots forms in the
microcirculation

• As platelets and clotting factors are consumed to

form microthrombi, coagulation fails

• Result of excessive clotting is bleeding

 Medical Management
• Most important management issue is treating the
underlying cause

• Secondary goal: correct secondary effects of

tissue ischemia by improving oxygenation,
replacing fluids, correcting electrolyte
imbalances, and administering vasopressor
medications

• Heparin infusion
 Disorders Associated with the
Lymphatic System
• LYMPHOMAS
– Hodgkin’s Disease
– Non – Hodgkin’s Disease

Hodgkin’s Disease Non – Hodgkin’s Disease

 Hodgkin’s Disease
• a relatively rare malignant B lymphocyte cell disease of the
lymph system that has an impressive cure rate

• more common in men

• a type of lymphoma
• has two peaks of incidence
 early 20’s
 after 50’s

• More treatable than non-Hodgkin’s

 Lymphoma
A. Hodgkin’s lymphoma
- Proliferation of Reed-Sternberg cells in a single lymph node
then travel to other lymph nodes
- Caused by Epstein Barr virus
- Progression:
a. Stage 1: Single lymph node
b. Stage 2: 2 or more lymph nodes on the same side of the
diaphragm or in an extralymphatic organ
(GOOD PROGNOSIS)
c. Stage 3: Both sides of diaphragm
d. Disease disseminates , spreads to other extralympathic
organs like spleen (POOR PROGNOSIS)
 Hodgkin’s Disease

• unicentric in origin
• Epstein – Barr virus
• REED – STERNBERG CELL
– malignant cell of Hodgkin’s
Disease
– pathologic hallmark
 Hodgkin’s Disease
• CLINICAL MANIFESTATIONS
– painless enlargement of one or more lymph
nodes (neck)
– painless, firm but not hard
 Hodgkin’s Disease
• CLINICAL MANIFESTATIONS
– pruritus
– cough, pulmonary effusion, jaundice, abdominal
pain, bone pain
– herpes zoster infection
– B SYMPTOMS
fever without chills, drenching sweats, weight loss >10%
– mild anemia
– absent / decreased reaction to sensitivity tests
 Medical Management
• Staging laparotomy then radiation therapy- obtain biopsy
specimen of retroperitoneal lymph nodes and both lobes
of liver and remove spleen

• Chemotherapy - 80% effective

• Radiation therapy is still very useful to extensive

adenopathy
Late manifestation:
 Hepatomegaly

 Spleenomegaly

 difficulty of breathing

 facial edema

 enlargement of the
lower extremities
 Non – Hodgkin’s Disease
• heterogeneous group of cancers
• originate from the neoplastic growth
of lymphoid tissues
• lymphoid tissues involved are largely
infiltrated with malignant cells
• spread of malignant lymphoid cells is
unpredictable
 Non- Hodgkins Lymphoma
Spread is unpredictable
Extranodal tissues
May involve B lymphocytes and T lymphocytes
↑ in age, ↑ incidence of NHL
 Etiologic Factor

Unknown
Immunodeficiencies
Viral infection
Incidence is ↑ in whites
Exposure to dyes, pesticides and solvent
 Pathophysiology
• NHL arises when some
lymphocytes begin to
divide uncontrollably and
grow in an abnormal way,
or when the cells do not
die as new lymphocytes
are produced to take their
place.
 Non – Hodgkin’s Disease
• CLINICAL MANIFESTATIONS
– symptoms may be absent or very minor
• early stage
– Lymphadenopathy
• stage III or IV
– “B symptoms”
(drenching night sweats, recurrent
fever, unintentional weight loss
>10%)
 Non – Hodgkin’s Disease
• MANAGEMENT
– radiation
– chemotherapy + radiation therapy
– bone marrow transplant
 Multiple Myeloma
Abnormal proliferation of plasma cells
Immature and malignant and invade the bone marrow,
lymph nodes and liver, spleen and kidneys
Leads to bone destruction throughout the body
Causes: Environmental, Genetic
Bone demineralization occurs and large amounts of Ca
are lost urine in blood and urine- renal calculi- renal
failure
Bence Jones CHON in urine, Increased BUN, creatinine,
increased calcium
• Risk Factors:
– familial tendency
– radiation therapy
– exposure to chemicals

• Clinical Manifestations:
– backache or bone pain
– sudden pathologic fracture
– diffuse osteoporosis
– hypercalcemia
• Diagnostic Test:
– bone marrow biopsy
– blood and urine examination
– (+) Bence Jones Protein

• Management:
1. Combination therapy
2. Bone marrow transplant
3. Reduce calcium level
4. Encourage activity