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. This is because of significant differences in the rates of hemolysis and associated diseases and because 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 use in our clinical practice setting. We also review the parameters present in diagnostic testing that allow 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 test results, a relatively infrequent but challenging situation, in order to assist in the overall evaluation spectrum for these patients. (Blood. 2017; 129(22):2971-2979

A Retrospective Study of the Combination of Rituximab, Cyclophosphamide and Dexamethasone for the Treatment of Relapsed/Refractory Warm Antibody Autoimmune Hemolytic Anemia

“…However, fewer than 20% of patients have a long-term response to corticosteroid therapy alone [4]. The recommended second-line therapy is splenectomy or rituximab [5, 6]. Rituximab is favored over splenectomy in patients with secondary WAIHA due to the low response rates to splenectomy in this patient population [7].…”

Autoimmune Haemolytic Anaemia in Patients with Cancer Diagnoses

“…Autoimmune haemolytic anaemia (AIHA) is a heterogeneous group of diseases characterized by autoantibody production to red cell antigens causing shortened survival of the red cells. Anaemia caused by red blood cell (RBC) autoantibodies is not common [1]. In 1946 it was observed that the red cells of patients with acquired haemolytic anaemia sometimes had a positive direct antiglobulin test (DAT) but patients with hereditary haemolytic anaemias were DAT negative and this led to the discovery of AIHA due to the development of autoantibody to the red cells [2].…”

“…AIHA has an incidence of 1-3 cases per 100,000/year [1]. More than half the cases of AIHA are warm AIHA with an incidence of 1/50,000 -80,000 persons [3,4].…”

Mixed IgM cold and IgG warm autoimmune hemolytic anemia complicated by acral gangrene

“…Presence of cold agglutinin in this case was clinically significant leading to gangrene with subsequent amputation. Treatment of WAIHA includes steroids with or without rituximab and or splenectomy with anticipated response in 80% of patients while treatment of CAD is more challenging . The treatment of mixed‐type AIHA is less well defined because of its rarity.…”