Current Approaches for the Treatment of Autoimmune Hemolytic Anemia
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REVIEW
Current Approaches for the Treatment of Autoimmune Hemolytic Anemia Jose´ Carlos Jaime-Pe´rez • Marisol Rodrı´guez-Martı´nez Andre´s Go´mez-de-Leo´n • Luz Tarı´n-Arzaga • David Go´mez-Almaguer
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Received: 28 September 2012 / Accepted: 26 April 2013 Ó L. Hirszfeld Institute of Immunology and Experimental Therapy, Wroclaw, Poland 2013
Abstract Autoimmune hemolytic anemia (AIHA) is an infrequent group of diseases defined by autoantibody mediated red blood cell destruction. Correct diagnosis and classification of this condition are essential to provide appropriate treatment. AIHA is divided into warm and cold types according to the characteristics of the autoantibody involved and by the presence of an underlying or associated disorder into primary and secondary AIHA. Due to its low frequency, treatment for AIHA is largely based on small prospective trials, case series, and empirical observations. This review describes in detail the different treatment approaches for autoimmune hemolytic anemia. Warm antibody type AIHA should be treated with steroids, to which most patients respond, although relapse can occur and maintenance doses are frequently required. Splenectomy is an effective second line treatment and can provide long-term remission without medication. Rituximab is a useful alternative for steroid refractory patients, those requiring high maintenance doses and unfavorable candidates for surgery. Promising therapeutic modifications with this monoclonal antibody are emerging including drug combinations, lower doses, and long-term use. Primary cold agglutinin disease has been recognized as having a J. C. Jaime-Pe´rez M. Rodrı´guez-Martı´nez A. Go´mez-de-Leo´n L. Tarı´n-Arzaga D. Go´mez-Almaguer Internal Medicine Division, Department of Hematology, ‘‘Dr. Jose´ Eleuterio Gonza´lez’’ University Hospital of the School of Medicine of the Universidad Auto´noma de Nuevo Leo´n, Monterrey, Mexico J. C. Jaime-Pe´rez (&) Servicio de Hematologı´a, Edificio ‘‘Dr. Rodrigo Barraga´n’’, 2 piso, Hospital Universitario ‘‘Dr. Jose´ E. Gonza´lez’’ Avenida Madero y Gonzalitos S/N, Colonia Mitras Centro, CP 64460 Monterrey, Nuevo Leo´n, Mexico e-mail: [email protected]
lymphoproliferative monoclonal origin. It is unresponsive to both steroids and splenectomy. Rituximab is currently the best therapeutic alternative for this condition, and several treatment regimens are available with variable responses. Keywords Hemolytic anemia Autoimmune Cold agglutinin disease Treatment Rituximab
Introduction Autoimmune hemolytic anemia (AIHA) is an infrequent group of diseases defined by autoantibody mediated red blood cell (RBC) destruction. It affects mostly, but not exclusively, adults, with an estimated annual incidence of 1–3/100,000 persons (Bottiger and Westerholm 1973; Gehrs and Friedberg 2002; Klein et al. 2010). RBC destruction is associated with the presence of immunoprotein molecules on the erythrocyte surface, including immunoglobulins, most frequently IgG, with antibody activity directed against s
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