Contributions
Abstract: PB1846
Type: Publication Only
Background
Autoimmune hemolytic anemia (AIHA) is characterized by red blood cell destruction mediated with autoantibodies against to RBC antigens. Most common type is warm AIHA which can be either idiopathic or secondary to underlying disorders with immun disturbance. Determining the optimal therapy is a challenge because of insufficient data from prospective controlled trials.
Aims
To evaluate the clinical charactheristics, treatment responses and outcomes of our AIHA patients.
Methods
The clinical data of 32 patients with AIHA diagnosed and treated in our center between 2008 and 2016 were retrospectively analyzed.
Results
Median age at diagnosis of AIHA was 45 years (range:20-74). Male/female ratio was 1/1.3. 24 of 32 patients (75%) had primary AİHA and 8 (25%) had secondary AIHA with underlying disorders as SLE in 2 patients,, mixed connection tissue disease (MCTD) in 2, psoriatic arthritis in 1, chronic lymphocytic leukemia (CLL) in 1, marginal zone lymphoma in 1 and, chronic HCV infection in 1. Median Hemoglobin (Hb) level was 7.4 gr/dl and 5 patients also had thrombocytopenia (<150000) beside hemolytic anemia. Mean LDH level was 544, indirect bilirubin was 2.7, reticulocyte was 11.3%. 18/32 patients (56%) required transfusion. In all patients who required treatment (94%) corticosteroids were the first-line therapy with an initial response rate of 93%. Median steroid duration was 3 months range between 1.5 to 96 months. Relapse was occured in 15 of 30 patients who recieved steroid (50%) with the median time to relapse (TTR) of 12 months (range:5-72 months). 11/30 patients (37%) required second-line therapy; seven had undergone splenectomy, three recieved rituximab, and one recieved danasin. All of the patients who undergone splenectomy had CR in first month and relapse after splenectomy was seen in 5/7 patients (71%) with a median duration of 60 months. Of three patients who were treated with standard dose of Rituximab; two achieved CR and one did not achieve any response. One of two rituximab-responded patients relapsed at 26. and 60. months and re-treated by rituximab; still following with CR for 16 months.
Conclusion
Although corticosteroids are the first choice of initial treatment of AİHA, most of the patients relapse at follow up. Steroid dependency and intolerance are also challenging. Splenectomy is still a considerable option for second-line therapy because of its high response rates and long remission durations. Rituximab is the other effective second-line therapy option with similar response rates to splenectomy. Until prospective studies will be performed, retrospective data would help the clinicians to choose best treatment algorithm for AIHA.
Session topic: 27. Enzymopathies, membranopathies and other anemias
Keyword(s): Splenectomy, Rituximab, Autoimmune hemolytic anemia (AIHA)
Abstract: PB1846
Type: Publication Only
Background
Autoimmune hemolytic anemia (AIHA) is characterized by red blood cell destruction mediated with autoantibodies against to RBC antigens. Most common type is warm AIHA which can be either idiopathic or secondary to underlying disorders with immun disturbance. Determining the optimal therapy is a challenge because of insufficient data from prospective controlled trials.
Aims
To evaluate the clinical charactheristics, treatment responses and outcomes of our AIHA patients.
Methods
The clinical data of 32 patients with AIHA diagnosed and treated in our center between 2008 and 2016 were retrospectively analyzed.
Results
Median age at diagnosis of AIHA was 45 years (range:20-74). Male/female ratio was 1/1.3. 24 of 32 patients (75%) had primary AİHA and 8 (25%) had secondary AIHA with underlying disorders as SLE in 2 patients,, mixed connection tissue disease (MCTD) in 2, psoriatic arthritis in 1, chronic lymphocytic leukemia (CLL) in 1, marginal zone lymphoma in 1 and, chronic HCV infection in 1. Median Hemoglobin (Hb) level was 7.4 gr/dl and 5 patients also had thrombocytopenia (<150000) beside hemolytic anemia. Mean LDH level was 544, indirect bilirubin was 2.7, reticulocyte was 11.3%. 18/32 patients (56%) required transfusion. In all patients who required treatment (94%) corticosteroids were the first-line therapy with an initial response rate of 93%. Median steroid duration was 3 months range between 1.5 to 96 months. Relapse was occured in 15 of 30 patients who recieved steroid (50%) with the median time to relapse (TTR) of 12 months (range:5-72 months). 11/30 patients (37%) required second-line therapy; seven had undergone splenectomy, three recieved rituximab, and one recieved danasin. All of the patients who undergone splenectomy had CR in first month and relapse after splenectomy was seen in 5/7 patients (71%) with a median duration of 60 months. Of three patients who were treated with standard dose of Rituximab; two achieved CR and one did not achieve any response. One of two rituximab-responded patients relapsed at 26. and 60. months and re-treated by rituximab; still following with CR for 16 months.
Conclusion
Although corticosteroids are the first choice of initial treatment of AİHA, most of the patients relapse at follow up. Steroid dependency and intolerance are also challenging. Splenectomy is still a considerable option for second-line therapy because of its high response rates and long remission durations. Rituximab is the other effective second-line therapy option with similar response rates to splenectomy. Until prospective studies will be performed, retrospective data would help the clinicians to choose best treatment algorithm for AIHA.
Session topic: 27. Enzymopathies, membranopathies and other anemias
Keyword(s): Splenectomy, Rituximab, Autoimmune hemolytic anemia (AIHA)