Chemotherapy/
Biotherapy for
Hematology Disease
Processes
Hematology Disease
Processes
 Sickle
Cell Anemia
 Immune Thrombocytopenia Purpura
 Autoimmune Hemolytic Anemia
 Paroxysmal Nocturnal
Hemoglobinuria
Sickle Cell Anemia
 Hemoglobinopathy
 Genetic
Disease
 Presence of Hemoglobin S, alone or
in combination with another
abnormal hemoglobin form.
 Red blood cell is sickle shaped with a
shortened life span
Incidence and Etiology of
Sickle Cell Anemia
 Most
common genetic disorder in
the United States (Hgb SS)
 Approximately 72,000 cases in
United States
Morbidity Associated
With Sickle Cell Anemia
 Infection
 Vaso-occlusive
crisis
 Acute chest syndrome
 Splenic Sequestration
 Aplastic Crisis
 Cerebral Vascular Accidents
Chronic Complications
with Sickle Cell Anemia
 Cardiac
and Pulmonary Changes
 Cholelithiasis
 Avascular Necrosis
 Renal Impairments
 Delayed Growth and Maturation
 Impaired Cognition
Immune Thrombocytopenia
Purpura
 Decrease
in platelets
 Caused by an immune mediated
response
 Classified as acute vs. chronic
 Often follows viral illness
 Can be drug-induced
 Chronic ITP often associated with
immune disorders
Incidence and Etiology
 Equal
incidence between boys and
girls
 Average age 1-10yo
 Increase incidence in the winter
months
Clinical Presentation
 Bruising, petechiae, with
or without
purpura
 Oral or gingival bleeding
 Epistaxis
 Menorrhagia
 Hematuria
 Gastrointestinal Bleeding
 Recent history of viral illness
Autoimmune Hemolytic
Anemia
 Decrease
hemoglobin due to red
blood cell destruction
 Immune mediated response
 Can be intravascular or extravascular
in orgin
Incidence and Etiology
 No
Known Cause
 Incidence is approximately 1 case per
80,000
 Can be associated with other
autoimmune conditions (SLE)
Clinical Presentation









Pallor, jaundice
Fatigue or irritability
Tachycardia, palpitations
Dyspnea
Dizziness
Headache
Splenomegaly
Hepatomegaly
Mental impairment, poor concentration,
chance in LOC
Paroxysmal Nocturnal
Hematuria
 Acquired
life threatening hematologic
disorder
 Triad of hemolytic anemia,
pancytopenia, and thrombosis
 Non-malignant clonal hematopoietic
stem cell disorder
Incidence and Etiology
 Approximately
8000-10,000 cases in
North America and Western Europe
 Predominately an adult disease
occurring between 30-45 years of
age
 10% of cases occur in patients less
than 21 years of age
 Males and females equally affected
Clinical Presentation of
PNH
 Fatigue
 Abdominal
pain
 Hematuria
 Hemolysis
 Thrombosis
 Bone
Marrow Failure
 Dysphagia
References
 Essentials
of Pediatric
Hematology/Oncology Nursing: A
Core Curriculum, 3rd Edition, 2008
Editor: Nancy Kline
 The Pediatric Chemotherapy and
Biotherapy Curriculum, 2nd Edition,
2007 Editor: Nancy Kline