The Child with

Hematologic
or Immunologic
Dysfunction

ERNIE B. GUEVARRA, M.D.

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Assessment of
Hematologic Function
• Complete blood count
• History and assessment findings
• Child’s energy and activity level
• Growth patterns

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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

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Classification of Anemias
• Etiology and physiology
– RBC and/or Hgb depletion
• Morphology
– Characteristic changes in RBC size,
shape, and/or color

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

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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
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Diagnostic Evaluation
• CBC
– Decreased RBCs
– Decreased Hbg and Hct
• Other tests for particular type of anemia

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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

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Nursing Considerations
• Prepare child and family for laboratory
tests
• Decrease oxygen demands
• Prevent complications
• Support family

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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

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Iron Deficiency Anemia
(cont’d)
• Pathophysiology
• Therapeutic management
• Prognosis
• Nursing considerations

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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

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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)

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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

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

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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

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Thalassemia
• Inherited blood disorders of hemoglobin
synthesis
• Classified by Hgb chain affected and by
amount of effect
• Autosomal recessive with varying
expessivity
– Both parents must be carriers to have
offspring with disease

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Pathophysiology
• Anemia results from defective synthesis of Hgb,
structurally impaired RBCs, and shortened life of
RBCs
• Chronic hypoxia
– Headache, irritability, precordial and bone
pain, exercise intolerance, anorexia,
epistaxis
• Detected in infancy or toddlerhood
– Pallor, FTT, hepatosplenomegaly, severe
anemia (Hgb <6)

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Thalassemia
∀α-Thalassemia
– Alpha chains affected
– Occurs in Chinese, Thai, African, and
Mediterranean people
∀β-Thalassemia
– Occurs in Greeks, Italians, and
Syrians
– β is most common and has four forms
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β-Thalassemia
• Four types
– Thalassemia minor—asymptomatic silent
carrier
– Thalassemia trait—mild microcytic anemia
– Thalassemia intermediate—moderate to
severe anemia + splenomegaly
– Thalassemia major (“Cooley’s anemia”) —
severe anemia requiring transfusions to
survive

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Diagnosis
• By hemoglobin electrophoresis
• RBC changes often seen by 6 weeks of
age
• Child presents with severe anemia, FTT

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Medical Management
• Blood transfusion to maintain normal Hgb
levels
• Side effect—hemosiderosis
• Treat with iron chelating drugs such
as deferoxamine (Desferal)
– Bindsexcess iron for excretion
by kidney

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Nursing Management
• Observe for complications of transfusion
• Emotional support to family
• Encourage genetic counseling
• Parent and patient teaching for self-care

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Prognosis
• Retarded growth
• Delayed or absent secondary sex
characteristics
• Expect to live well into adulthood with
proper clinical management
• Bone marrow transplant is potential cure

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Aplastic Anemia
• All formed elements of the blood are
simultaneously depressed:
“pancytopenia”
• Hypoplastic anemia: profound depression
of RBCs but normal WBCs and platelets

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Aplastic Anemia (cont’d)
• Etiology
– Primary (congenital)
– Secondary (acquired)
• Diagnostic evaluation
• Therapeutic management
• Nursing consideration

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

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Types of Hemophilia
• Hemophilia A
– “Classic hemophilia”
– Deficiency of factor VIII
– Accounts for 80% of cases of
hemophilia
– Occurrence: 1 in 5000 males

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Types of Hemophilia
(cont’d)
• Hemophilia B
– Also known as Christmas disease
– Caused by deficiency of factor IX
– Accounts for 15% of cases of
hemophilia

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Etiology of Hemophilia A
• X-linked recessive trait
• Males are affected
• Females may be carriers
• Degree of bleeding depends on amount of
clotting factor and severity of a given injury
• Up to ⅓ of cases have no known family history
– In these cases disease is caused by a NEW
mutation

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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

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Manifestations (cont’d)
• 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

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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

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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
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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

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

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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

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

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ITP Forms
• Acute self-limiting
– Often follows URI or other infection
• Chronic (>6 months duration)

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ITP
• Diagnostic evaluation
• Therapeutic management
• Prognosis
• Nursing considerations

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Disseminated Intravascular
Coagulation (DIC)
• Disorder of coagulation that occurs as
complication of numerous pathologic
processes
– Hypoxia
– Acidosis
– Shock
– Endothelial damage

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Pathophysiology of DIC
• Coagulation process is abnormally
stimulated
• Excessive amounts of thrombin are
generated
• Fibrinolytic mechanisms are activated
and cause extensive destruction of
clotting factors

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DIC
• Diagnostic evaluation
• Therapeutic management
• Nursing considerations

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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)

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Nursing Considerations—
Epistaxis
• Remain calm, keep child calm
• Have child sit up and lean forward
• Pressure to nose
• Further evaluation if bleeding continues

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

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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

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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

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

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

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Chromosome Studies
• Children with Trisomy 21:20X greater risk
for developing ALL
• Children with more than 50 chromosomes
on the leukemic cells have the best
prognosis
• Specific translocations of chromosomes
on leukemic cells can affect prognosis

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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

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Pathophysiology (cont’d)
• 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

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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

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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

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Therapeutic Management
• Chemotherapeutic agents
• Cranial irradiation (in some cases)

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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

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

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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

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Infection Control
• Environment
• Hand hygiene
• Visitor restriction

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Managing
Chemotherapeutic Agents
• “Vesicants”—sclerosing agents even in
minute amounts
• Interventions for extravasation
• Risk for anaphylaxis

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Managing Problems
of Drug Toxicity
– Nausea/vomiting
– Anorexia
– Mucosal ulceration
– Neuropathy
– Hemorrhagic cystitis
– Alopecia
– Mood changes
– Moon face

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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

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Lymphomas
• Hodgkin disease
– More prevalent in 15-19 yrs of age
• Non-Hodgkin lymphoma (NHL)
– More prevalent in children <14 yrs of
age

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

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Four Types of Hodgkin
Disease
• Lymphocytic predominance
• Nodular sclerosis
• Mixed cellularity
• Lymphocytic depletion
• Accurate staging is basis for treatment
and prognosis

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Classifications of Hodgkin
Disease
• Classification A: asymptomatic
• Classification B: temperature of 38°C or
higher for three consecutive days, night
sweats, unexplained wt loss of 10% or
more over previous 6 mos

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

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Therapeutic Management
• Radiation
• Chemotherapy (alone or with radiation)
• Prognosis
• Nursing considerations

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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

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NHL (cont’d)
• Diagnostic evaluation
• Therapeutic management
• Prognosis
• Nursing consideration

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Immunologic Deficiency
Disorders
• HIV/AIDS
– Epidemiology
– Etiology and pathophysiology
– Clinical manifestations
– Diagnostics
– Therapeutic management

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Blood Transfusion Therapy
• Verify identity of recipient and verification
of donor’s blood group
• Monitor VS
• Use appropriate filter
• Use blood within 30 minutes of arrival
• Infuse over 4 hours maximum

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Transfusion Reactions
• Hemolytic: the most severe, but rare
• Febrile reactions: fever, chills
• Allergic reaction: urticaria, pruritis, laryngeal
edema
• Air emboli: may occur when blood is transfused
under pressure
• Hypothermia
• Electrolyte disturbances: hyperkalemia from
massive transfusions or pt with renal problems

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Nursing Responsibilities
• Identify donor and recipient blood types
and groups before transfusing
• Transfuse slowly for first 15-20 minutes
• Observe carefully for patient response
• Stop transfusion immediately if
signs/symptoms of transfusion reaction;
notify practitioner

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Delayed Reactions to Blood
Transfusion
• Transmission of infection
– Hepatitis, HIV, malaria, syphilis, other
– Blood banks test vigorously and
discard units of infected blood
• Delayed hemolytic reaction
– Destruction of RBCs and fever 5-10
days after transfusion
– Observe for posttransfusion anemia
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Family Concerns
• Difficult decision for HTSC transplant
– Child faces death without transplant
– Preparing child for transplant places
child at great risk
• No “rescue” procedure if complications
follow HTSC transplants
• Nursing considerations

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Hematopoietic Stem Cell
Transplantation (HSCT)
• Used to establish healthy cells in both
malignant and nonmalignant disease
• Ablative therapy: high-dose combination
chemo (with or without radiation) to
eradicate unhealthy cells and suppress
immune system to prevent rejection of
transplanted marrow

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HSCT (cont’d)
• Stem cells harvested from bone marrow,
peripheral blood, or umbilical vein of
placenta
• Stem cells given to patient by IV
transfusion
• Newly transfused stem cells repopulate
ablative bone marrow

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Allogeneic Bone
Marrow Transplant (BMT)
• Involves matching histocompatible donor
with recipient
• Limited by presence of suitable marrow
donor

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Umbilical Cord Blood Stem
Cell Transplantation
• Rich source of hematopoietc stem cells
for use in children with cancers
• Stem cells found with high frequency in
circulation of newborns
• Benefit of umbilical cord blood is blood’s
relative immunodeficiency at birth,
allowing for partially matched unrelated
cord blood transplants to be successful

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Autologous BMT
• Uses patient’s own marrow collected from
disease-free tissue, frozen and
sometimes treated to remove malignant
cells
• Has been used to treat neuroblastoma,
Hodgkin disease, NHL, Wilms' tumor,
rhabdomyosarcoma, and Ewing sarcoma

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Peripheral Stem Cell
Transplants
(PSCT)
• A type of autologous transplant
• Different type of collection from patient
• Stimulate production of high number of stem
cells then collect stem cells by an “apheresis”
machine
• Stem cells separated from whole blood
• Remaining blood cells and plasma returned to
patient after apheresis
• Stem cells frozen for later transfusion to patient

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