Autoimmune hemolytic anemia

Abstract

The diagnosis of autoimmune hemolytic anemia (AIHA) can be made with a stepwise approach that aims to identify laboratory and clinical evidence of hemolysis and then determine the immune nature of hemolysis with the direct anti-globulin test. Once alternative causes for these findings have been excluded, AIHA is established, and the clinician must search for secondary causes, as well as identify the type of AIHA. Rituximab is now the preferred second-line treatment for primary warm AIHA and first-line treatment for primary cold agglutinin disease (CAD), either as monotherapy or combined with bendamustine. Complement inhibitors have shown utility in stabilizing AIHA patients with acute severe hemolysis. Future prospects are discussed and include the C1s inhibitor BIVV009 (sutimlimab) that is now entering phase 3 studies for CAD.

Conflict of interest statement

Conflict-of-interest disclosure: A.H. and Q.A.H. have received honoraria from Bioverativ.

© 2018 by The American Society of Hematology. All rights reserved.

Figures

Figure 1.

Blood film appearances in patients with AIHA (both using May Grunwald Giemsa stain). (A) Spherocyte in a patient with warm AIHA (original magnification, ×100). (B) Red cell agglutination in a patient with cold agglutinin disease (×40).

Figure 2.

Diagnostic pathway for AIHA. DAggT, direct agglutination test; DIIHA, drug-induced immune hemolytic anemia; HA, hemolytic anemia; HDN, hemolytic disease of the newborn; HTR, hemolytic transfusion reaction; PLS, passenger lymphocyte syndrome; RT, room temperature. *The final diagnosis of CHAD or mixed AIHA is based on the overall clinical picture, including supportive serological findings. †For example, the thermal amplitude. **Saline-suspended red cells and patient’s serum at room temperature for 30 to 60 minutes. Adapted from Hill et al with permission.