Hemolytic anemia due to warm autoantibodies.
The diagnosis of autoimmune hemolytic anemia (AHA) requires evidence of shortened red blood cell (RBC) survival mediated by autoantibodies directed against autologous RBCs. About 80 percent of patients with AHA have warm-reactive antibodies of the IgG isotype; the remainder exhibit cold-reactive autoantibodies. Typical patients exhibit anemia, reticulocytosis, spherocytes and polychromasia on the blood film and a positive direct antiglobulin test (DAT). Increased indirect serum bilirubin, urinary urobilinogen and serum lactate dehydrogenase (LDH), and decreased serum haptoglobin are not required for the diagnosis, but are frequently present. Patients with AHA and no underlying associated disease are said to have primary or idiopathic AHA. AHA in patients with associated autoimmune disease and certain malignant or infectious diseases is classified as secondary. The etiology of AHA is unknown. Patients with symptomatic anemia require transfusion of RBCs. Prednisone and splenectomy may provide long term remission. Rituximab, intravenous immunoglobulin, immunosuppressive drugs and danazol have been effective in refractory cases and for patients who are poor candidates for surgery.
Packman
Charles H
CH
University of North Carolina School of Medicine, Chapel Hill, North Carolina, United States. cpackman@carolinas.org
D000743:Anemia, Hemolytic / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D001323:Autoantibodies / Q000009:adverse effects*; D001803:Blood Transfusion; D003298:Coombs Test; D006801:Humans; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D013102:Spherocytes; D013156:Splenectomy; D013696:Temperature
D001323:Autoantibodies; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents