The treatment of autoimmune hemolytic anemia with complement inhibitor iptacopan: a case report

Abstract

Autoimmune hemolytic anemia (AIHA) is a type of hemolytic anemia. In this condition, the body produces anti-red blood cell autoantibodies due to immune dysfunction. This results in accelerated destruction of red blood cells. According to the best temperature for autoantibodies to stick to red blood cells, there are three types: warm antibody type (wAIHA), cold antibody type (cAIHA), and mixed warm-cold antibody type (mAIHA). This article presents a case of acute severe warm antibody autoimmune hemolytic anemia in an elderly male patient. The patient exhibited symptoms including jaundice of the skin, mucous membranes, and urine with a soy sauce color. Laboratory tests were as follows: hemoglobin (HGB) as low as 31 g/L; indirect bilirubin (IBIL) as high as 162 μmol/L; lactate dehydrogenase (LDH) level as high as 1,295 IU/L; reticulocyte percentage (RET%) > 4%; Coombs test positive; conjugated beads protein assay < 0.2 g/L; direct anti-human globulin test positive; indirect anti-human globulin test positive; anti-IgG+++; anti-C3d negative; cold agglutinin test (CAT) negative. After admission, the patient was given a combination of two treatments: methylprednisolone and immunoglobulin. During the treatment, the patient developed a hemolytic crisis. He was immediately given iptacopan and high-dose glucocorticoid shock therapy. After treatment, the patient’s hemolytic-related symptoms improved rapidly. The hemoglobin levels remained within a safe range after stopping the blood transfusion. It is reported as follows.