Autoimmune haemolytic anaemias: A retrospective study of 93 patients

Abstract

Introduction: Autoimmune haemolytic anaemia (AIHA) is an infrequent and heterogeneous disease in its pathophysiology and clinical behaviour, therefore it is generally managed empirically.

Patients and methods: We conducted an observational, retrospective and multicentre study of 93 patients diagnosed with AHAI in 9 Spanish hospitals between 1987 and 2017, with a median follow-up of 28 months.

Results: Median age of 67 years; 85% AHAI for hot antibodies and 64% primary AHAI. The lowest haemoglobin values at diagnosis related to patients under 45 years of age and serological type IgG+C. Of the patients, 92% received first line treatment, 54% second line, and 27% third line. The warm AHAI were treated in first line with steroids, with overall responses of 83% and complete of 58%. Rituximab in monotherapy or in association with steroids was administered to 34 patients with overall responses close to 100% (complete responses 40-60%), relegating splenectomy to the third line. The immunosuppressive treatment was administered in patients with autoimmune diseases or in corticoid-dependent patients.

Discussion: We found high rates of response to steroids, with very prolonged treatments that cause side effects and corticoid dependence in a third of patients. The combination of steroids with rituximab in the first line, could be indicated in patients with low levels of haemoglobin and serological type IgG+C. The high relapse rates make necessary the development of randomised studies with new drugs or the combination with existing ones, which allow longer response times and with fewer side effects.

Keywords: Anemia hemolítica autoinmune; Autoimmune haemolytic anaemia; Corticoides y anemia hemolítica autoinmune; Corticosteroids and autoimmune haemolytic anaemia; Rituximab and autoimmune haemolytic anaemia; Rituximab y anemia hemolítica autoinmune; Tratamiento de anemia hemolítica autoinmune; Treatment of autoimmune haemolytic anaemia.