All issues > Volume 41(6); 1998

Original Article

J Korean Pediatr Soc. 1998;41(6):775-784. Published online June 15, 1998.

Immunomodulation Therapy in Aplastic Anemia : Relapse Rate, Complications and Long-term Survival During Follow-up for More than 1 Year

Jun Ah JA Lee¹, Hyoung Jin HJ Kang¹, Hyo Jeong HJ Han¹, Hyoung Soo HS Choi¹, Eun Sun ES Yoo¹, Hee Young HY Shin¹, Joong Gon JG Kim¹, Hyo Seop HS Ahn¹

¹Department of Pediatrics, Seoul National University College of Medicine Seoul, Korea

Correspondence Hyo Seop HS Ahn ,Email: 1

Abstract

Purpose
: We evaluated the response to immunomodulation therapy, long-term survival and relapse rate of aplastic anemia during follow-up for more than 1 year.
Methods
: Twenty-eight children, with moderate to severe aplastic anemia were followed for more than one year and 7 children expired after therapy, were analyzed. Antilymphocyte globulin(ALG) or antithymocyte globulin(ATG) by itself was given to 27 patients, and cyclosporine A(CsA) combined with ALG was given to 8 patients. ALG(ATG) was administered for 8 days in 20mg/kg/day when used alone, and for 5 days in 10mg/kg/day when combined with CsA. CsA was orally administered on the 14th day till 180th day of therapy, with 5mg/kg/day for first 14 days and 3mg/kg/day thereafter.
Results
: Fifteen out of 35 patients(43%) showed a response. Median interval to response was 3 month(1-40 months). Response rate to ALG＋CsA was 50%, compared to 41% in ALG(or ATG) alone. The interval of aplastic anemia symptoms to treatment showed a tendency to be shorter in responders(4.1±2.2 months) than in non-responders(17.5±6.4 months). Relapse occurred in 3 of 15 responders(20%). Evolution to secondary clonal hematologic disorders was not observed. Overall actuarial survival at 3 year was 78.2%
Conclusion
: The results of immunomodulation therapy suggest that response would be better for patients whose interval from symptoms of aplastic anemia to treatment is short. Our results also suggest that ALG＋CsA would be a better treatment modality to improve response rate.

Keywords :Aplastic anemia, Immunomodulation therapy, Antilymphocyte globulin, Antithymocyte globulin, Cyclosporine A