Etiology and therapy of thrombocytopenia associated with sarcoidosis

Abstract

Moderate to severe thrombocytopenia as a complication of sarcoidosis occurs with sufficient frequency to indicate that sarcoidosis may predispose to the development of thrombocytopenia. This report describes the clinical course and laboratory findings of two black adolescent boys with sarcoidosis who developed severe thrombocytopenia. An analysis of the findings in these patients, together with a review of an additional 37 reported cases, indicates that patients with sarcoidosis and thrombocytopenia can be divided into three groups: In Group A a moderate reduction of platelets is part of a generalized suppression of peripheral blood elements in association with proved or suspected portal hypertension; in Groups B and C moderate to severe thrombocytopenia is probably secondary to a platelet destructive phenomenon. The bleeding in Group B patients is mild and of long duration, and in Group C patients, bleeding is fulminant and associated with a high mortality rate unless treated with corticosteroids and/or splenectomy. Although the evidence is indirect, the occurrence of severe thrombocytopenia in patients with sarcoidosis may be an expression of a generalized autoimmune phenomenon.