Pediatric Hematology Lecture
Pediatric Hematology Lecture
Pediatric Hematology Lecture
or Immunologic Dysfunction
Chapter 26
Assessment of
Hematologic Function
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
Therapeutic
Management
Nursing Considerations
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
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
Medical Management
Aggressive treatment of infection
Possible prophylactic antibiotics from 2 mos-5 yrs
Medical Management
(contd)
Medical Management
(contd)
Nursing Management
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
Manifestations of
Hemophilia
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
Manifestations of
Von Willebrand Disease
Easy bruising
Epistaxis
Gingival bleeding
Excessive bleeding with lacerations or surgeries
Menorrhagia
Diagnosis
Laboratory Findings
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
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
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
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
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
Diagnostic Evaluation
Based on history, physical manifestations
Peripheral blood smear
Immature leukocytes
Frequently low blood counts
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
Reverse isolation
Risks of HSCT
Prognosis
If relapse after HSCT: dismal prognosis
Identified factors for determining prognosis
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
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)
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