[Progress in diagnosis and treatment of autoimmune hemolytic anemia]

In 2017, The British Society of Haematology published new guidelines for the diagnosis and management of autoimmune hemolytic anemia (AIHA). Usually this is diagnosed using a combination of clinical and laboratory findings of hemolysis using the direct antiglobulin test (DAT). The revised guidelines propose several steps to evaluate and diagnose patients with unexplained hemolysis and a negative DAT, and they recommend screening for cold agglutinin disease (CAD) using a direct agglutination test (DAggT) before evaluating the cold agglutinin titer. Rituximab is becoming the preferred second-line choice for steroid-refractory warm AIHA and the first-line choice for primary CAD. Rituximab is an off-label drug for use in AIHA treatment in Japan, but in future will be used as standard therapy. Anti-C1s antibody is a new drug for CAD that has entered phase 3 trials.