TY - CHAP M1 - Book, Section TI - Hemolytic Anemia Resulting from Immune Injury A1 - Packman, Charles H. A2 - Kaushansky, Kenneth A2 - Lichtman, Marshall A. A2 - Prchal, Josef T. A2 - Levi, Marcel M. A2 - Press, Oliver W. A2 - Burns, Linda J. A2 - Caligiuri, Michael PY - 2015 T2 - Williams Hematology, 9e AB - SUMMARYAutoimmune hemolytic anemia (AHA) is characterized by shortened red blood cell (RBC) survival and the presence of autoantibodies directed against autologous RBCs. Demonstration of antibody and/or complement on RBC membranes, usually by a positive direct antiglobulin test (DAT, also referred to as the Coombs test) is essential for diagnosis. Most patients with AHA (80 percent) exhibit warm-reactive antibodies of the immunoglobulin (Ig) G isotype on their red cells. Most of the remainder of patients exhibit cold-reactive autoantibodies. Two types of cold-reactive autoantibodies to RBCs are recognized: cold agglutinins and cold hemolysins. Cold agglutinins are generally of IgM isotype, whereas cold hemolysins usually are of IgG isotype. The DAT may detect IgG, proteolytic fragments of complement (mainly C3), or both on the RBCs of patients with warm-antibody AHA. In cold-antibody AHA, only complement is detected because the antibody dissociates from the RBCs during washing of the cells. About half of patients with AHA have no underlying associated disease; these cases are termed primary or idiopathic. Secondary cases are associated with underlying autoimmune, malignant, or infectious diseases or with ingestion of certain drugs.Although most patients do not require transfusion of RBCs, transfusion should not be withheld from those with symptomatic anemia. In warm-antibody AHA, rituximab and glucocorticoids are effective in slowing the rate of hemolysis. Splenectomy is indicated for patients who are refractory to medical therapy or who require an unacceptably high maintenance dose or prolonged administration of glucocorticoids. Intravenous immunoglobulin may provide short-term control of hemolysis. Immunosuppressive drugs and danazol have been used successfully in refractory cases. In cold agglutinin- and cold hemolysin-mediated hemolysis, keeping the patient warm and treating underlying lymphoproliferative disorders usually are effective. Rituximab has been effective in about half of cases of cold AHA. Drug-induced immune hemolytic anemia usually is ameliorated by discontinuation of the offending drug. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - accessmedicine.mhmedical.com/content.aspx?aid=1121094665 ER -