Haema 2013; 4(1):31-46
by Flora Kοntopidou1, Helen Sinni2, Theodoros P. Vassilakopoulos2
12nd Clinic of Internal Medicine National and Kapodistrian University of Athens, Ippokration Hospital, Αthens,
2Hematology Clinic National and Kapodistrian University of Athens, Laiko General Hospital, Goudi, Αthens, Greece
AIHA is an uncommon but potentially fatal condition. It results from autoantibodies with speciﬁcity against red blood cell (RBC) antigens leading to the premature removal of red cells from the circulation. Anemia may occur if the rate of red cell removal exceeds the ability of the bone marrow to produce new red cells. AIHA may be idiopathic or secondary to drugs or underlying conditions such as lymphoproliferative disease, viral or mycoplasmal infection, or autoimmune disorders. Cases of AIHA generally are classified according to the characteristic temperature reactivity of the RBC autoantibody and include warm AIHA, cold agglutinin syndrome, paroxysmal cold hemoglobilinuria and mixed-type AIHA. The diagnosis is based on the clinical presentation and a serologic work up which Includes the documentation of the presence of hemolysis along with demonstration of the presence of an autoantibody and/or complement components on the surface of the patient’s red cells [usually by the direct antiglobulin (Coombs’) test]. The aim of treatment of AIHA is to reverse the hemolytic process either by reducing antibody production or by removing the primary site of destruction, or by reducing the interac- tion between splenic macrophages and the antibody-coated RBCs. Unfortunately, the evidence available for the treatment of AIHA is sparse and of low methodologic quality, based predominantly on small case series. Glucocorticoids are frequently used as the first therapy for warm AIHA. Splenectomy is the most commonly described treatment for relapsed or refractory AIHA. Other effective agents include rituximab and immunomodulatory drugs. The most useful single therapy in cold agglutinin disease is avoidance of cold exposure, while rituximab (with or without cytotoxic treatment) forms the basis of drug therapy, when such intervention is required.. Packed red cell transfusions are used only when AIHA presents as a medical emergency in patients with underlying cardiac disease.