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KMID : 0338420020170030204
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The Korean Journal of Internal Medicine
2002 Volume.17 No. 3 p.204 ~ p.206
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Allogeneic Bone Marrow Transplantation in Shwachman-Diamond Syndrome with Malignant Myeloid Transformation -A Case Report-
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Moon Hee Lee/So Young Park
Min Byoung Chae/Yee Gyung Kwack/Moon Hee Lee/In Ho Kim/Young Soo Kim/Chul Soo Kim
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Abstract
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Shwachman-Diamond syndrome (SDS) is a rare genetic disorder of unknown pathogenesis involving exocrine pancreatic insufficiency and hematological and skeletal abnormalities. About 25% of patients develop hematopoietic malignancies. We report on a case of acute myeloid leukemia (M2) in a 21-year-old woman affected by SDS. She was treated with conventional chemotherapy (idarubicin plus cytarabine) and reached complete remission of leukemia. After induction chemotherapy, she underwent allogeneic bone marrow transplantation (BMT). The BMT preparative regimen consisted of total body irradiation (TBI) followed by cyclophosphamide. Cyclosporin A and short term methotrexate were used for graft-versus-host disease prophylaxis. After a follow-up of 12 months, she is alive leukemia free off any immunosuppressive agent. Although experience in this field is scarce, we speculate that bone marrow failure in SDS is an indication for BMT which is the only curative treatment option.
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KEYWORD
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Shwachman-Diamond syndrome, Bone marrow transplantation, Acute myeloid leukemia,
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