Mortality in immune thrombocytopenic purpura: report of seven cases and consideration of prognostic indicators

Abstract

ITP (immune thrombocytopenic purpura) is generally considered a benign disease. Despite what may be severe thrombocytopenia, most patients with ITP do not suffer significant bleeding episodes. Mortality is rare, and the majority of cases are managed successfully with conventional treatments. For patients who maintain a platelet count of > 20,000/microliters, aggressive immunosuppressive therapy is usually unwarranted. There are some patients with ITP who are at increased risk of serious morbidity and mortality from their disease. Fatal cases of ITP are rarely mentioned in published reports. In this study we review the incidence of mortality and describe the deaths of seven patients with ITP, with the aim of identifying and improving management of high-risk cases. Based on our review of the literature and analysis of cases at our institution, fatal cases of ITP fall into three groups: death from intracranial hemorrhage with severe thrombocytopenia, death after splenectomy, and death due to infection after cytotoxic treatments. Patients who appear to carry increased risk include: 1) older patients; 2) chronic, refractory patients with a history of hemorrhage; and 3) patients with concomitant bleeding diatheses such as uremia and hemophilia.