Autoimmune hemolytic anemia: classification and therapeutic approaches

Abstract

Autoimmune hemolytic anemia (AIHA) is a relatively uncommon cause of anemia. Classifications of AIHA include warm AIHA, cold AIHA (including mainly chronic cold agglutinin disease and paroxysmal cold hemoglobinuria), mixed-type AIHA and drug-induced AIHA. AIHA may also be further subdivided on the basis of etiology. Management of AIHA is based mainly on empirical data and on small, retrospective, uncontrolled studies. The therapeutic options for treating AIHA are increasing with monoclonal antibodies and, potentially, complement inhibitory drugs. Based on data available in the literature and our experience, we propose algorithms for the treatment of warm AIHA and cold agglutinin disease in adults. Therapeutic trials are needed in order to better stratify treatment, taking into account the promising efficacy of rituximab.

Keywords::

• autoimmune hemolytic anemia
• chronic cold agglutinin disease
• drug-induced hemolysis
• paroxysmal cold hemoglobinuria
• red blood cells transfusion
• rituximab

Financial & competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Writing assistance was utilized in the production of this manuscript. The authors would like to thank M-A Charrier for her administrative assistance.

Notes

AIHA: Autoimmune hemolytic anemia; ALPS: Autoimmune lymphoproliferative syndrome.

Data from [1,17].

APTT: Activated partial thromboplastin time; Ig: Immunoglobulin.