Management of adult immune thrombocytopenia.
Immune thrombocytopenia (ITP) is a heterogeneous disease with regard to pathogenesis, severity, spontaneous course and response to treatment. Except in patients with severe bleeding tendency and very low platelet counts (< 10 x 10(9)/L), there are no clear rules on the indications for treatment. The standard initial therapy is corticosteroids, but the optimal dose and duration of therapy is unknown and in practice, some patients may be overtreated by aiming for complete remission (CR). In patients who have no sustained response after steroids, the most effective single therapy is splenectomy. Laparascopic splenectomy has a very low mortality and moderate morbidity. Preoperative prediction of success is difficult. About 50% of patients are in CR or partial remission after 5 years, but there are few data on the long-term outcome. Patients who fail steroids and splenectomy are difficult to treat. The choice may be palliative, with low doses of steroids or aggressive therapy with the intention of sustained remission. In selected patients, high-dose immunoglobulin or anti-D may be useful to temporarily raise the platelet count. Other drugs tried in ITP had either no or very limited clinically meaningful efficacy.