Otoimmun Hemolitik Anemilerin Steroid ve Steroid Dışı Tedavilere Yanıtının Değerlendirilmesi

Abstract

Evaluation of the response of Autoimmune Hemolytic Anemia to steroid and non-steroidal therapies, Hacettepe University Faculty of Medicine, Thesis of İnternal Medicine Department. Ankara 2018 İntraduction and Purpose: Autoimmun hemolytic anemia , as a result of patient produce antibodies against its own erythrocytes and anemia is a disease characterized by the breakdown of erythrocytes. OİHA is divided into cold and hot types. İt is classified as idiophatic and secondary according to its etiology. Steroids are the first-choice drugs to treat. Alternating agents or splenectomy are used in cases with unresponsive or recurrence. Patients and Methods: This study is a retrospective cohort study included patients with autoimmune hemolytic anemia with positive Coombs test positive for all causes between 2010 and 2017. Criteria for inclusion in the study were those aged 18 and over, who were diagnosed with OİHA ( with direct Coombs test + ), received steroid therapy, were not responding to steroids, were on secondary medications and were treated with splenectomy.Complete response to steroid and other treatments (CR) was defined as normalization of Hb>12 g/dl , hemolysis markers , and partial response Hb 10-12 g/dl , normalitazion of hemolysis markers within 3 months treatment. Results: The study population consisted of 68 patients (95.8%) with hot type OİHA and 3 patients (4.2%) with cold type OİHA. 42.3% of the patients were male and the mean age was 52.7 ± 17.1 years. 35.2% (n: 25) of the patients did not respond to steroid treatment. Secondary etiology rate was higher in patients who did not respond to steroid compared to responders (68% versus 19.6%; p <0.001). The rate of response to steroid treatment in the primary OİHA is 82.2% and it is 34.6% in secondary OİHA (p <0.001). The relapse rate in the whole population was 18.3% (n: 13). All patients who had relapse not responding to steroid treatment showed a response after splenectomy. Responding to steroid treatment, 88.9% of patients with relapse responded after splenectomy. 41.7% of patients who did not respond to steroid treatment received Rituximab, 25% MMF, 16.7% cyclosporine, 8.3% cyclosofamide and 8.3% IVIG. As a result; It should be evaluated by considering the etiology in steroid treatment in OİHA. Immunsuppressive therapy may be associated with etiology and type of OİHA in the response rate. However, cases with splenectomy are more stable. It may be important to determine the predictors that can predict the treatment response with prospective and large sample studies.