The Child with Hematologic

or Immunologic Dysfunction
Chapter 26

Assessment of
Hematologic Function

Complete blood count

History and assessment findings
Childs energy and activity level
Growth patterns

Anemia
The most common hematologic disorder of
childhood
Decrease in number of RBCs and/or hemoglobin
concentration below normal
Decreased oxygen-carrying capacity of blood

Classification of Anemias
Etiology and physiology
RBC and/or Hgb depletion

Morphology
Characteristic changes in RBC size, shape, and/or
color

Consequences of Anemia
Decrease in oxygen-carrying capacity of blood
and decreased amount of oxygen available to
tissues
When anemia develops slowly, child adapts

Effects of Anemia
on Circulatory System
Hemodilution
Decreased peripheral resistance
Increased cardiac circulation and turbulence
May have murmur
May lead to cardiac failure

Cyanosis
Growth retardation

Diagnostic Evaluation
CBC
Decreased RBCs
Decreased Hbg and Hct

Other tests for particular type of anemia

Therapeutic
Management

Treat underlying cause

Transfusion after hemorrhage if
needed
Nutritional intervention for deficiency
anemias
Supportive care
IV fluids to replace intravascular
volume
Oxygen
Bed rest

Nursing Considerations

Prepare child and family for laboratory tests

Decrease oxygen demands
Prevent complications
Support family

Iron Deficiency Anemia

Caused by inadequate supply of dietary iron
Generally preventable
Iron-fortified cereals and formulas for infants
Special needs of premature infants
Adolescents at risk due to rapid growth and poor
eating habits

Iron Deficiency Anemia

Most common nutritional problem in US
Caused by any number of factors that
decrease the supply of Fe, impair its
absorption, increase the bodys need
for Fe, or affect the synthesis of
hemoglobin.
Large milk intake with low solid food
source
Rapid growth
Inadequate diet, menses, impaired
absorption

Iron Deficiency Anemia

Pathophysiology facts
Birth- full term infant
Fe supply=300mg

-During the last

trimester-Fe transfer
from
mom @ rate
of 4mg/day
Maternal Fe stores
are adequate for up
to 4-6 months
If diet doesnt
supplement the
maternal/fetal
stores

Iron Deficiency Anemia

Clinical Manifestations
Milk baby-overweight
Milk is a poor source of iron,
Vit.C, zinc & Flouride
Pale, porcelain-like skin
Underweight
Poor muscle dev,edema,
retarded growth,
Irritable, tachycardic, fatigue,
koilonychia
Social and cognitive abilities

Iron Deficiency Anemia

Mgmt/Nursing

Prevention/Screening/Teaching
Dietary counseling
Iron Fortified formula/cereal up to 12 m
Iron rich foods upon solids
Fe supplementation (Ferrous sulfate)
-give b/w meals/Vit C to absorption
-Keep no more than 1m supply=toxic

Sickle Cell Anemia

A hereditary hemoglobinopathy
Ethnicity
Occurs primarily in blacks
Occurrence 1 in 375 infants born in US
1 in 12 have sickle cell trait
Occasionally also in persons of
Mediterranean descent
Also seen in South American, Arabian,
and East Indian descent

Etiology of Sickle Cell

In areas of world where malaria is common,
individuals with sickle cell trait tend to have
survival advantage over those without trait
Autosomal recessive disorder
1 in 12 blacks are carriers (have sickle cell
trait)
(If both parents have trait, each offspring
will have 1 in 4 likelihood of having disease)

Pathophysiology
Partial or complete replacement of normal Hgb
with abnormal hemoglobin S (Hgb S)
Hemoglobin in the RBCs takes on an elongated
sickle shape
Sickled cells are rigid and obstruct capillary blood
flow
Microscopic obstructions lead to engorgement and
tissue ischemia
Hypoxia occurs and causes sickling

Sickled Hemoglobin
Under conditions of dehydration, acidosis,
hypoxia, and temperature elevations, HgbS
changes its structure in the cell membrane from
a pliable disk to a crescent or sickle shaped RBC.
Sickling response is reversible with oxygenation
& hydration

Pathophysiology
Large tissue infarctions occur
Damaged tissues in organs; impaired function
Splenic sequestration
May require splenectomy at early age

Prognosis
No cure (except possibly bone marrow
transplants)
Supportive care/prevent sickling
episodes
Frequent bacterial infections may occur
due to immunocompromise
Bacterial infection is leading cause of
death in young children with sickle cell
disease
Strokes in 5%-10% of children with
disease
Result in neurodevelopmental delay,
mental retardation

Systems Affected

Sickle Cell Crisis

Precipitating factors
Anything that increases bodys need for oxygen or
alters transport of oxygen
Trauma
Infection, fever
Physical and emotional stress
Increased blood viscosity due to dehydration
Hypoxia
From high altitude, poorly pressurized airplanes,
hypoventilation,vasoconstriction due to
hypothermia

Sickle Cell Crisis (contd)

Acute exacerbations that vary in severity and
frequency
Types
Vaso-occlusive [VOC] thrombotic
Most common type of crisisvery painful
Stasis of blood with clumping of cells in
microcirculationischemiainfarction
Signs: fever, pain, tissue engorgement

Sickle Cell Crisis (contd)

Types (contd)
Splenic sequestration
Life threateningdeath can occur within hours
Blood pools in the spleen
Signs
Profound anemia, hypovolemia, and shock

Diagnosis of Sickle Cell

Cord blood in newborns
Newborn screening done in 43 states
Genetic testing to identify carriers and children
who have disease
Sickle-turbidity test
Quick screening purposes in children >6 mos of age

Medical Management
Aggressive treatment of infection
Possible prophylactic antibiotics from 2 mos-5 yrs

Medical Management
(contd)

Monitor reticulocyte count regularly to evaluate

bone marrow function
Blood transfusion, if given early in crisis, may
reduce ischemia

Medical Management
(contd)

Frequent transfusion decreases hemosiderosis

(iron in tissues)
Treat with iron-chelation such as feroxamine +
vitamin C to promote iron excretion

Nursing Management

Monitor childs growthwatch for failure to thrive

Careful multi-system assessment
Assess pain
Observe for presence of inflammation or possible
infection
Carefully monitor for signs of shock

Psychosocial Needs

Coping mechanisms
Support with genetic counseling
Financial needs
Caregiver role strain
Living with chronic illness in the family

Hemophilia
A group of hereditary bleeding disorders that
result from deficiencies of specific clotting factors

Hemophilia
X linked recessive disorder
Carried on female chromosome; males are
affected
Deficiency of Factor 8, produced by the
liver, necessary for the formation of
thromboplastin for clotting

Types of Hemophilia
Hemophilia A

Classic hemophilia
Deficiency of factor VIII
Accounts for 80% of cases of hemophilia
Occurrence: 1 in 5000 males

Hemophilia A-Severity
Degrees
Severe-Spontaneously bleed w/o trauma
Moderate-Bleed with trauma
Mild-severe trauma/surgery

Types of Hemophilia
(contd)
Hemophilia B

Also known as Christmas disease

Caused by deficiency of factor IX
Accounts for 15% of cases of hemophilia

Manifestations of
Hemophilia

Bleeding tendencies range from mild to

severe
Symptoms may not occur until 6 mos of age
Mobility leads to injuries from falls and
accidents
Hemarthrosis
Bleeding into joint spaces of knee, ankle,
elbow leading to impaired mobility
Ecchymosis

Manifestations (contd)
Epistaxis
Bleeding after procedures
Minor trauma, tooth extraction, minor surgeries
Large subcutaneous and intramuscular hemorrhages
may occur
Bleeding into neck, chest, mouth may compromise
airway

Clinical Therapy
Can be diagnosed through amniocentesis
Genetic testing of family members to identify
carriers
Diagnosis on basis of hx, labs, and exam
*Labs: Low levels of factor VIII or IX, prolonged PTT
*Normal: platelet count, PT, and fibrinogen

Medical Management
DDAVP
IV
Causes 2-4 X increase in factor
VIII activity
Used for mild hemophilia
Replace missing clotting factors
Transfusions
At home with prompt intervention
to reduce complications
Following major or minor
hemorrhages

Prognosis
Historically, most died by 5 yrs age
Now mild to moderate hemophilia patients live
near normal lives
Gene therapy for future
Infused carrier organisms act on target cells to
promote manufacture of deficient clotting factor

Interventions
Close supervision and safe
environment
Dental procedures in controlled
situation
Shave only with electric razor
Superficial bleedingapply
pressure for at least 15 minutes
+ ice to vasoconstrict
If significant bleeding occurs,
transfuse for factor replacement

Managing Hemarthrosis
During bleeding episodes, elevate and immobilize
joint
Ice
Analgesics
ROM after bleeding stops to prevent contractures
PT
Avoid obesity to minimize joint stress

Von Willebrand Disease

A hereditary bleeding disorder, involving
deficiency of Von Willebrand factor, (a
plasma protein, and the carrier for
factor VIII)
Von Willebrand factor is necessary for
platelet adhesion
Transmitted as autosomal dominant trait
Occurs in males and females
Gene for disease is located on
chromosome 12

Manifestations of
Von Willebrand Disease

Easy bruising
Epistaxis
Gingival bleeding
Excessive bleeding with lacerations or surgeries
Menorrhagia

Diagnosis
Laboratory Findings

Decreased Von Willebrand factor levels

Von Willebrand antigen levels
Decreased platelet agglutination
Prolonged bleeding time
PTT may be normal or prolonged

Treatment of
Von Willebrand Disease
Infusion of Von Willebrands protein concentrate
DDAVP infusion prior to surgery or to treat
bleeding episode (synthetic vasopressin)
Aminocaproic acid (Amicar) to treat bleeding in
mucous membranes (in some cases)

Interventions
Avoid aspirin or NSAIDs (increased bleeding time
and inhibit platelet function)
Manage bleeding episodes with prompt infusion
therapy
Children with Von Willebrands have normal life
expectancy if well managed

Idiopathic
Thrombocytopenic Purpura
An acquired hemorrhagic disorder characterized
(ITP)
by
Thrombocytopenia: excessive destruction of
platelets
Purpura: discoloration caused by petechiae beneath
the skin

ITP Forms
Acute self-limiting
Often follows URI or other infection

Chronic (>6 months duration)

ITP

Diagnostic evaluation
Therapeutic management
Prognosis
Nursing considerations

Epistaxis (Nosebleeding)
Isolated and transient epistaxis is common in
childhood
Recurrent or severe episodes may indicate
underlying disease
Vascular abnormalities, leukemia, thrombocytopenia,
clotting factor deficiency diseases (Von Willebrand
disease and hemophilia)

Nursing Considerations
Epistaxis

Remain calm, keep child calm

Have child sit up and lean forward
Pressure to nose
Further evaluation if bleeding continues

Neoplastic Disorders
Leading cause of death from disease in children
past infancy
Almost half of all childhood cancers involve blood
or blood-forming organs

Leukemias
Most common form of childhood cancer
3-4 cases per 100,000 caucasian children <15 yrs
old
More frequent in males >1 yr old
Peak onset between 2 and 6 yrs of age
Survivability

Classification of
Leukemias

Leukemia: A broad group of malignant disease of

bone marrow and lymphatic system
Complex disease with varying heterogeneity
Classifications are increasingly complex

Morphology
Acute lymphoid leukemia (ALL)
Acute non-lymphoid (myelogenous) leukemia
(ANLL or AML)
Stem cell or blast cell leukemia

Symptoms
ALL: lymphatic, lymphocytic, lymphoblastic, and
lymphoblastoid leukemia
AML: granulocytic, myelocytic, monocytic,
myelogenous, monoblastic, and non-myeloblastic
leukemia

Prognosis

ALL-**initial WBC count**(<50,000)

**age**@ DX ( >2&<10y=good)
**Cytogenetics (chromosomal makeup)
**Sex (males get testicular relapse, testes
resistant to chemo)
**Immunologic subtype
**ALL better than AML

Pathophysiology
Leukemia is an unrestricted proliferation of
immature WBCs in the blood-forming tissues of
the body
Liver and spleen are the most severely affected
organs

Pathophysiology (contd)
Although leukemia is an overproduction of WBCs,
often acute form causes low leukocyte count
Cellular destruction takes place by infiltration and
subsequent competition for metabolic elements

Consequences of
Leukemia

Anemia from decreased RBCs

Infection from neutropenia
Bleeding tendencies from decreased platelet
production
Spleen, liver, and lymph glands show marked
infiltration, enlargement, and fibrosis

Diagnostic Evaluation
Based on history, physical manifestations
Peripheral blood smear
Immature leukocytes
Frequently low blood counts

LP to evaluate CNS involvement

Bone marrow aspiration or biopsy

Mgmt./Follow Protocols
Chemotherapeutic agents
Cranial irradiation (in some cases)

Mgmt./Follow Protocols

Chemotherapy
(antineoplastic
agents)
Radiotherapy
Immunotherapy
4 phases:
Induction
Intensification/co
nsol.
CNS prophylax
Maintenance
BMT:
allogeneic/autologo
us

Prepare families for dx/tx

procedures

Provide cont emotional

support

Reverse isolation

FYI=BMA center of the

bone is hollow and
contains the cells that later
become working blood
cells or leukemic cells

Four Phases of Therapy

Induction therapy: 4-6 weeks
CNS prophylactic therapy: intrathecal
chemotherapy
Intensification (consolidation) therapy: To
eradicate residual leukemic cells and prevent
resistant leukemic clones
Maintenance therapy: to preserve remission

Hematopoietic Stem Cell

Transplantation (HSCT)

Donors may be relatives or non-relatives

Antigen-matched or mismatched
Peripheral stem cells may be used
Stem cells from umbilical cord blood

Risks of HSCT

Significant risk of morbidity and mortality

Graft vs. host disease (GVHD)
Overwhelming infection
Severe organ damage
Cure after HSCT: up to 60%-70%

Prognosis
If relapse after HSCT: dismal prognosis
Identified factors for determining prognosis

Initial WBC count

Age at time of diagnosis
Type of cell involved
Gender
Karyotype analysis

Nursing Considerations

Assessment
Nursing diagnosis
Planning
Implementation
Prepare child and family for procedures
Pain management
Prevent complication of myelosuppression

Increased Susceptibility
to Infection
At time of diagnosis and relapse
During immunosuppressive therapy
After prolonged antibiotic therapy that
predisposes to the growth of resistant organism

Infection
Control
Nursing
Care
Environment
Hand hygiene
Visitor restriction

Nursing
Care
Infection
Control
Manage problemsprovide favored foods
of irradiation and
drug toxicity = mouth care, soft toothbrush,
mouthwash, hydrogen
N/V, anorexia,
peroxide, saline
mucosal
Vincristine, numbness temp
ulceration,
temporary, wigs, hats
neuropathy,
hemorrhagic
decrease Na intake, look
cystitis, alopecia, healthy
moon face, mood analgesics
changes, pain

Managing
Chemotherapeutic Agents
Vesicantssclerosing agents even in minute
amounts
Interventions for extravasation
Risk for anaphylaxis

Nursing Diagnoses
Risk for injury related to malignant process,
treatment
Risk for fluid volume deficit related to nausea,
vomiting
Altered nutrition
Impaired skin integrity
Altered family processes
Fear related to diagnosis, procedures,
treatments

Lymphomas
Hodgkin disease
More prevalent in 15-19 yrs of age

Non-Hodgkin lymphoma (NHL)

More prevalent in children <14 yrs of age

Hodgkin Disease
Neoplastic disease originating in lymphoid system
Often metastasizes to spleen, liver, bone marrow,
lungs, and other tissues

Diagnostics
Clinical manifestations of Hodgkin disease
Lymph node biopsy for diagnosis and staging
Presence of Reed-Sternberg cells is characteristic
of Hodgkin disease

Therapeutic
Management

Radiation
Chemotherapy (alone or with radiation)
Prognosis
Nursing considerations

Non-Hodgkin Lymphoma
Approximately 60% of pediatric lymphomas
are NHL
Clinical appearance
Disease usually diffuse rather than nodular
Cell type undifferentiated or poorly
differentiated
Dissemination occurs early, often, and
rapidly
Mediastinal involvement and invasion of
meninges

Immunologic Deficiency
Disorders
Severe Combined Immunodeficiency
Syndrome (SCID) and Wiskott-Aldrich
Syndrome-the body is unable to mount an
immune response.
The immune systems function is to
recognize self from nonself and to
initiate a response to eliminate the
nonself or antigen.
Cell surface markers=body cells with
specific cell surface markers unique to the
individual

Protective Mechanisms
Intact skin
Saliva, sweat,
tears, stomach
acids
Sneezing,
coughing
Primary lymphoid
organs
(thymus,BM,liver)
Secondary
lymphoid organs
(spleen, lymph
nodes, GALT)

Vertical Transmission
(Perinatal)

HIV + mother to her newborn

Antepartum
Intrapartum:@ delivery
Postpartum: via breastfeeding-minimal in U.S.,
carries a risk as high as 28%

Nursing Responsibilities
Its the Law
MUST counsel & offer testing to those who
appear at delivery with NO record of an HIV test
during pregnancy
If a woman declines HIV testing, a signed
objection MUST be attempted
Offer testing to a child, whose mother has not
been tested
Childs HIV status is strictly confidential, schools
may not have info unless parents allow

Therapeutic Mgmt.
Assess compliance
Assuring adherence to med. schedule
Promoting weight gain-clinical improvements
include growth retardation improvements,
decrease hepatospleno, encephalopathy, and
immune sys. Fx.
Nutritional mgmt to help FTT