Thelansis

Cold agglutinin disease (CAD), also known as primary or idiopathic CAD, is a clonal B-cell lymphoproliferative disorder of the bone marrow that results in autoimmune hemolytic anemia (AIHA). CAD predominantly affects elderly or middle-aged people. IGHV4-34 almost exclusively encodes CAD antibodies; in rare instances, anti-I cold agglutinins may also be encoded by IGHV3 family genes. Cold agglutinins associated with CAD are monoclonal and most often of the immunoglobulin M (IgM) class with κ light-chain restriction. After an IgM cold agglutinin binds to its antigen on the erythrocyte surface, complement is activated by the classical pathway, and complement activation is essential for hemolysis. Most effective therapies have aimed to target the pathogenic B-cell clone. Currently, however, novel complement-directed therapies are being rapidly developed.   The estimated incidence of Cold agglutinin disease (CAD) varies 0.8 to 3 per 100,000 population per year, and the prevalence is 15 ...