Treatment of autoimmune hemolytic anemia at the departement of hematology of the UMC Ljubljana in the period from 2000–2011

Abstract

Background: Autoimmune hemolytic anemia (AIHA) is an acquired hemolytic anemia and can be treated as an idiopathic or primary disease or can be associated with some other hematologic diseases (secondary). AIHA is a disease characterized by the presence of auto-antibodies directed against antigens on the red cell membrane, rare can the result of Coombs test be negative although there are all signs of AIHA present. Depending on the temperature at which auto-antibodies bind to the surface of an erythrocyte, we talk about AIHA with warm agglutinins and AIHA with cold agglutinins. Treatment is diverse as are also the causes of AIHA. The most important thing is to decrease the production of antibodies or to prevent the erythrocyte from decomposing with bound antibodies and to treat the disease symptomatically. Patients and methodes: With a retrospective analysis we searched through database of 70 patients with idiopathic (90 %) and secondary (10 %) autoimmune hemolytic anemia with warm (84.3 %) and cold (10 %) type agglutinin, who were treated at the Hematologic Department of the UMC Ljubljana during the period from 2000–2011. I processed the data with the SPSS program system. Results: Methylprednisolone was the first choice of treatment in 92. 5 % patients with primary and secondary AIHA with response rate of 79 % and 70.9 % complete remission rate. 22.7 % of the patients with complete remission after corticosteroids treatment , relapsed during the period of time and received a second-line treatment with cyclosporine (87 % response rate), rituximab or underwent splenectomy. Another attempt was made with methylprednisolone. The patients, who were refractory to the initial methylprednisolone treatment or required a higher maintenance dose, were treated in second line with cyclosporine (69 % response rate), rituximab (57 % responsee rate). One patient was treated successfully with mycophenolate mofetil and two patients, who were refractory to all other treatments, achieved a stable state of disease with splenectomy. The effective treatment for patients with cold agglutinin AIHA were corticosteroids and rituximab. We achieved an improvement of the condition in secondary AIHA when we treated the underlying disease. 6 % of all patients were refractory to any kind of treatment. Conclusions: Treatment with glucocorticoids is the most efficient treatment for patients with AIHA with warm and cold agglutinin. If the patients are refractory to the first-line therapy, we switch to a second-line therapy, which depends on etiology and concomitant diseases. Patients who are resistant to standard available treatments generally present with severe complications.