Treatment of Autoimmune Hemolytic Anemia

  • Omar Niss
  • Russell E. WareEmail author


Autoimmune hemolytic anemia (AIHA) is a relatively uncommon disorder, characterized by autoreactive antibodies directed against endogenous red blood cell antigens. AIHA caused by warm-reacting antibodies is the most common form, and half of these are idiopathic. Excluding secondary causes of AIHA is the first step in treatment decision-making. Treatment of AIHA is also dictated by the type of causative antibody, patient’s age, and comorbidities. Warm AIHA responds well to corticosteroids initially, but a second-line, steroid-sparing treatment is commonly needed. Rituximab, splenectomy, and immunosuppressive medications are used as second-line treatment options in warm AIHA; however, the optimal order of these therapies is not yet known. Cold AIHA is usually part of an underlying illness or disease, and does not respond well to steroids or splenectomy; and rituximab is the first-line treatment in adult forms of monoclonal cold agglutinin disease. In this chapter, we discuss the treatment approaches to different forms of AIHA and emphasize differences between adult and pediatric AIHA. We also discuss novel treatment approaches, especially for specific forms of secondary AIHA, highlighting how unraveling the genetic mechanisms of AIHA can lead to more precise and effective therapies that avoid toxicities.


Autoreactive antibodies Idiopathic Primary Evans syndrome Common variable immune deficiency 


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© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Division of Hematology, Department of PediatricsCincinnati Children’s Hospital Medical Center and University of Cincinnati College of MedicineCincinnatiUSA

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