Treatment of idiopathic thrombocytopenic purpura (ITP)

In summary, idiopathic thrombocytopenic purpura (as defined here) should be treated initially with glucocorticoids unless there is life-threatening hemorrhage (such as intracranial bleeding), in which case emergency splenectomy is indicated, since it generally gives the most prompt improvement in platelet count of all modes of the disease and to better define the cause of the thrombocytopenia. A complete, sustained remission following treatment with steroids may occasionally be obtained. In most cases the steroid response is transient, and splenectomy is the most definitive measure with respect to a lifelong cure. Complete clinical cure is obtained in 70-80% of instances following splenectomy, with a very low operative risk. The remainder either fail to respond or experience relapse. In this group of patients steroids should again be tried; if a good response is obtained, steroids should be given every second or third day to lessen long-term side effects. If these measures fail, the use of immunosuppressive agents is justified. Both vinca alkaloids and cyclophosphamide are especially useful. Vinca alkaloids act more promptly, but cyclophosphamide may have a more lasting effect. Azathioprine may also be used; however, it appears to yield a lower remission rate. Nonsteroidal immunosuppressive agents should not be used as the primary modality of therapy. And, due to their carcinogenic and teratogenic potential, they should be avoided, if possible, especially in children and in women during their childbearing years.