Autoimmune hemolytic anemia in patients with ß-thalassemia major.

ABSTRACT

Hemolysis is a common feature in patients with ß-thalassemia major. As a result, autoimmune hemolytic anemia complicating ß-thalassemia is easily overlooked. Here, the authors described the clinical features and management of 7 patients with ß-thalassemia major and autoimmune hemolytic anemia. These patients had fever, cough, and tea-colored urine on admission. The laboratory investigations showed a significant drop in hemoglobin and increased serum bilirubin. Coombs' tests revealed that anti-immunoglobulin G (IgG) and anti-C3 was positive in 7 and 5 cases, respectively, whereas anti-Rh E alloantibody was positive in 3 cases. All the patients received corticosteroids treatments and blood transfusions. Patients with anti-Rh E alloantibodies also received immunoglobulin treatments. Six of the patients responded well to the management, but 1 patient developed recurrent autoimmune hemolytic anemia that required cyclosporin A treatment. All the patients remained well by following up for more than 6 months.