Autoimmune blood dyscrasias in five patients with hypogammaglobulinemia: response of neutropenia to vincristine.
Five patients with autoimmune blood dyscrasias and primary hypogammaglobulinemia are described. The autoimmune neutropenia in two patients rapidly responded to vincristine but was resistant to steroids. The three patients with autoimmune hemolytic anemia responded to steroids. Vincristine is therefore the drug of choice in patients with autoimmune neutropenia and hypogammaglobulinemia and is preferable to a trial of steroids in severely ill patients. Two of the patients had increased numbers of circulating T suppressor cells for in vitro immunoglobulin production. It is therefore unlikely that the autoimmunity in these patients is based on a lack of such suppressor cells.
D000293:Adolescent; D000328:Adult; D000361:Agammaglobulinemia / Q000150:complications / Q000276:immunology; D000380:Agranulocytosis / Q000188:drug therapy*; D001323:Autoantibodies / Q000096:biosynthesis; D001327:Autoimmune Diseases / Q000150:complications* / Q000276:immunology; D005260:Female; D006402:Hematologic Diseases / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D008875:Middle Aged; D009503:Neutropenia / Q000150:complications / Q000188:drug therapy* / Q000276:immunology; D009504:Neutrophils / Q000276:immunology; D013601:T-Lymphocytes / Q000276:immunology; D050378:T-Lymphocytes, Regulatory / Q000276:immunology; D014750:Vincristine / Q000627:therapeutic use*
D001323:Autoantibodies; D014750:Vincristine