Therapy for adults with refractory chronic immune thrombocytopenic purpura

Adult chronic immune thrombocytopenic purpura (ITP) is a common hematologic disorder; about 14 000 to 16 000 new cases occur each year in the United States. Initial treatment with corticosteroids and splenectomy results in normal or ''safe'' platelet counts in more than 70% of patients. Treatment of patients refractory to these two treatments is difficult. This paper describes a structured approach to therapy that is based on a literature review and personal experience, including experience with treatment of chronic ITP in special situations (such as emergent bleeding, pregnancy, and central nervous system bleeding). Treatment of most patients with chronic ITP is fairly straightforward, but management of patients refractory to corticosteroids and splenectomy can be difficult. Large, randomized studies are clearly needed to better evaluate the many types of treatment that are recommended for refractory patients.