How I treat autoimmune hemolytic anemia

Abstract

Autoimmune hemolytic anemia (AIHA) is an uncommon entity that presents diagnostic, prognostic and therapeutic dilemmas despite being a well-recognized entity for over 150 years. Because of significant differences in the rates of hemolysis as well as associated diseases and cause there is considerable clinical heterogeneity. In addition there is a lack of clinical trials required to refine and update standardized and evidence-based therapeutic approaches. To aid the clinician in AIHA management we present four vignettes that represent and highlight distinct clinical presentations with separate diagnostic and therapeutic pathways that we utilize in our clinical practice setting. We also review the parameters present in diagnostic testing that allows for prognostic insight and present algorithms for both diagnosis and treatment of the AIHA patient in diverse situations. This is done in the hope that this review may offer guidance in regard to personalized therapy recommendations. A section is included for the diagnosis of suspected AIHA with negative testing, a relatively infrequent but challenging situation in order to assist in the overall evaluation spectrum for these patients.