The Clinical Pictures of Autoimmune Hemolytic Anemia

Abstract

Autoimmune hemolytic anemia is characterized by shortened red blood cell survival and a positive Coombs test. The responsible autoantibodies may be either warm reactive or cold reactive. The rate of hemolysis and the severity of the anemia may vary from mild to severe and life-threatening. Diagnosis is made in the laboratory by the findings of anemia, reticulocytosis, a positive Coombs test, and specific serologic tests. The prognosis is generally good but renal failure and death sometimes occur, especially in cases mediated by drugs.

Keywords: Agglutinin; Autoimmunity; Direct antiglobulin test; Hemolysin; Hemolysis.

Fig. 1

Physical examination in warm antibody AIHA. Skin pallor, icterus and nailbed pallor in a patient with treatment-refractory warm AIHA, hemoglobin 4.8 g/dl (photographs courtesy of Professor A. Salama, with kind permission of the patient).

Fig. 2

Physical examination in cold antibody AIHA. a Livedo reticularis (photograph courtesy of Professor A. Salama). b Acrocyanosis involving the toes in a patient with a high-titer IgM cold agglutinin (reprinted from Sinha A, Richardson G, Patel RT : Cold agglutinin related acrocyanosis and paroxysmal hemolysis. Eur J Vasc Endovasc Surg, 30:563-565, copyright 2005, with permission from Elsevier).

Fig. 3

Blood films from patients with AIHA. Used with permission from Lichtman's Atlas of Hematology, www.accessmedicine.com. a Cold agglutinin disease, low power, slide made at room temperature. Note agglutination of RBCs. b Warm antibody AIHA. Note frequent small, round RBCs lacking central pallor, large bluish RBCs (polychromasia), Howell-Jolly body and nucleated RBCs.