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Autoimmune disorders and malignant lymphoma

Autoimmune disorders of the Coombs' positive hemolytic anemia (AIHA) and idiopathic thrombocytopenic purpura (ITP) types were found in 11 (2.1%) of 515 patients with the non‐Hodgkin's lymphomas. Four additional patients with Hodgkin's disease and similar disorders were included for analysis. In only two instances did the autoimmune disorder precede the lymphoma. AIHA was frequently associated with splenomegaly, constitutional symptoms, and, in eight of nine instances, far‐advanced lymphomas of the diffuse histiocytic and diffuse histiocytic‐lymphocytic types. In contrast, three of four patients who developed ITP had no evidence of active lymphoma at the onset of thrombocytopenia. The hemolysis of AIHA was most often controlled by splenectomy or corticosteroids with concurrent chemotherapy, but in all instances of either AIHA or ITP the prognosis clearly related to the underlying lymphoma and not the autoimmune disorder. No patient died of anemia, thrombocytopenia, or complications thereof. Based on this review, therapeutic guidelines for the management of these patients are proposed.