Immunomodulation Therapy in Aplastic Anemia: Relapse Rate, Complications and Long-term Survival During Follow-up for More than 1 Year.
- Author:
Jun Ah LEE
1
;
Hyoung Jin KANG
;
Hyo Jeong HAN
;
Hyoung Soo CHOI
;
Eun Sun YOO
;
Hee Young SHIN
;
Joong Gon KIM
;
Hyo Seop AHN
Author Information
1. Department of Pediatrics, Seoul National University College of Medicine Seoul, Korea.
- Publication Type:Original Article
- Keywords:
Aplastic anemia;
Immunomodulation therapy;
Antilymphocyte globulin;
Antithymocyte globulin;
Cyclosporine A
- MeSH:
Anemia, Aplastic*;
Antilymphocyte Serum;
Child;
Cyclosporine;
Follow-Up Studies*;
Humans;
Immunomodulation*;
Recurrence*
- From:Journal of the Korean Pediatric Society
1998;41(6):775-784
- CountryRepublic of Korea
- Language:Korean
-
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.