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 ,000 cases in North America and Western Europe Predominately an adult disease occurring between 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, 3 rd Edition, 2008 Editor: Nancy Kline The Pediatric Chemotherapy and Biotherapy Curriculum, 2 nd Edition, 2007 Editor: Nancy Kline