Progress in diagnosis and treatment of autoimmune hemolytic disorders

Discovery of the Coombs' test was an epoch-making event in the history of managing autoimmune hemolytic anemia (AIHA). The Coombs' test allows immune-related hemolytic anemia to be distinguished from nonimmune acquired hemolytic anemia, but also creates a complicated category; Coombs-negative AIHA. To resolve this problem, several trials have been conducted to detect immunoglobulin (Ig) G molecules on erythrocytes (RBC-IgG) that the Coombs' test cannot detect. In Japan, RBC-IgG can be quantitated using a radioimmunoassay, but this procedure is time-consuming and expensive. Convenient quantitative analysis of RBC-IgG has recently been reported using flow cytometry, a semi-quantitative method. In the treatment of warm AIHA, corticosteroids represent the first-line therapy. For refractory and relapsed cases, the choice may be between splenectomy and rituximab, which is becoming the preferred second-line treatment. Progress in the treatment of warm AIHA is also reviewed in this article.