Self-Paced Course CNE: The Autoimmune Cytopenias: Deactivating the Self-Destruct Sequence

Autoimmune cytopenias include autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP), autoimmune neutropenia (AIN), and Evan’s syndrome (ES). This diverse group of hematologic disorders is characterized by the production of autoantibodies by the patient’s own immune system against hematopoietic cells in the blood or bone marrow. Immune-mediated destruction of platelets, red cells or neutrophils can result in significant bleeding, anemia or infectious complications. Management focuses on choosing the least toxic approach to maintain a safe blood count. The majority of children who require therapeutic intervention respond to corticosteroids, immune globulin or growth factors. Patients with severe or refractory disease may require alternative therapies including cytotoxic agents, monoclonal antibodies or stem cell transplant. Review of several case studies will allow the nurse to contrast the diagnostic work-up and treatment options for these potentially life threatening diseases.