July 1981

Combined Sickle Cell Disease and Autoimmune Hemolytic Anemia

Author Affiliations

From the Departments of Medicine (Dr Chaplin), Preventive Medicine (Dr Chaplin), and Pediatrics (Dr Zarkowsky), Washington University School of Medicine, St Louis.

Arch Intern Med. 1981;141(8):1091-1093. doi:10.1001/archinte.1981.00340080127029

• Four patients experienced combined sickle cell disease and autoimmune hemolytic anemia within the past ten years. A fifth patient had positive direct antiglobulin test results without verified autoimmune hemolysis. Severely accelerated hemolysis was observed in four patients; anemia was severe, and the reticulocyte count rose into the 60% to 88% range. During the period of active autoimmune hemolysis, decline of the reticulocyte count into the 6% to 16% range was associated with rapid decrease in the hemoglobin level, requiring transfusion. All five patients were already alloimmunized by transfusions administered before onset of the autoimmune hemolytic anemia; two or more allospecificities were identified in four of five patients. The presence of autoantibody notably compromised compatibility testing; three patients experienced posttransfusion hemoglobinuria, and in vivo cross matching with 51Cr-labeled donor RBCs was employed on three occasions. All patients responded to corticosteroids; mercaptopurine was also administered to one patient. The direct antiglobulin test result reverted to negative in all patients after hospital discharge.

(Arch Intern Med 1981;141:1091-1093)