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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
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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