Chemotherapy/Chemotherapy/Biotherapy for Biotherapy for Hematology Disease Hematology Disease ProcessesProcesses

Hematology Disease Hematology Disease Processes Processes Sickle Cell AnemiaImmune Thrombocytopenia Purpura

Autoimmune Hemolytic Anemia

Paroxysmal Nocturnal Hemoglobinuria

Sickle Cell AnemiaSickle Cell Anemia

HemoglobinopathyGenetic DiseasePresence 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 Incidence and Etiology of Sickle Etiology of Sickle Cell AnemiaCell AnemiaMost common genetic disorder in the United States (Hgb SS)

Approximately 72,000 cases in United States

Morbidity Associated Morbidity Associated With Sickle Cell With Sickle Cell AnemiaAnemiaInfectionVaso-occlusive crisisAcute chest syndromeSplenic SequestrationAplastic CrisisCerebral Vascular Accidents

Chronic Chronic Complications with Complications with Sickle Cell AnemiaSickle Cell AnemiaCardiac and Pulmonary Changes

CholelithiasisAvascular NecrosisRenal ImpairmentsDelayed Growth and Maturation

Impaired Cognition

Immune Immune Thrombocytopenia Thrombocytopenia PurpuraPurpuraDecrease in plateletsCaused by an immune mediated response

Classified as acute vs. chronic

Often follows viral illnessCan be drug-inducedChronic ITP often associated with immune disorders

Incidence and Incidence and EtiologyEtiologyEqual incidence between boys and girls

Average age 1-10yoIncrease incidence in the winter months

Clinical PresentationClinical PresentationBruising, petechiae, with or without purpura

Oral or gingival bleedingEpistaxisMenorrhagiaHematuriaGastrointestinal BleedingRecent history of viral illness

Autoimmune Autoimmune Hemolytic AnemiaHemolytic AnemiaDecrease hemoglobin due to red blood cell destruction

Immune mediated responseCan be intravascular or extravascular in orgin

Incidence and Incidence and EtiologyEtiologyNo Known CauseIncidence is approximately 1 case per 80,000

Can be associated with other autoimmune conditions (SLE)

Clinical PresentationClinical PresentationPallor, jaundiceFatigue or irritabilityTachycardia, palpitationsDyspneaDizzinessHeadacheSplenomegalyHepatomegalyMental impairment, poor

concentration, chance in LOC

Paroxysmal Paroxysmal Nocturnal HematuriaNocturnal HematuriaAcquired life threatening hematologic disorder

Triad of hemolytic anemia, pancytopenia, and thrombosis

Non-malignant clonal hematopoietic stem cell disorder

Incidence and Incidence and EtiologyEtiologyApproximately 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 Clinical Presentation of PNHof PNHFatigueAbdominal painHematuriaHemolysisThrombosisBone Marrow FailureDysphagia

ReferencesReferencesEssentials 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