Fatal cyclophosphamide-induced congestive heart failure in a 10-year-old boy with Shwachman-Diamond syndrome and severe bone marrow failure treated with allogeneic bone marrow transplantation.
Fatal cyclophosphamide-induced congestive heart failure in a 10-year-old boy with Shwachman-Diamond syndrome and severe bone marrow failure treated with allogeneic bone marrow transplantation.
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一名患有 Shwachman-Diamond 综合征和严重骨髓衰竭的 10 岁男孩因环磷酰胺诱发致命性充血性心力衰竭，接受同种异体骨髓移植治疗。
DOI：
10.1097/00043426-199024000-00012
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发表时间：
1990
期刊：
影响因子：
--
通讯作者：
Lipton,JM
中科院分区：
文献类型：
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作者：
Tsai,PH;Sahdev,I;Herry,A;Lipton,JM
1例10岁男孩，患有Shwachman-Diamond综合征和严重骨髓衰竭，接受大剂量环磷酰胺、白消安和抗胸腺细胞球蛋白治疗，随后输注人类白细胞抗原相同的混合淋巴细胞培养（MLC）无反应同胞骨髓。他出现心律失常和顽固性低血压，于移植后第23天死亡。尸检结果与环磷酰胺诱导的全心炎一致。骨髓显示出早期植入的迹象。异基因骨髓移植可能是Shwachman-Diamond综合征伴严重骨髓衰竭的一种治疗选择。然而，致命的移植后心膜炎由于剂量的环磷酰胺通常不与心脏死亡可能是一个意想不到的问题。骨髓移植作为治疗这种综合征的进一步试验可能是必要的，可能使用较低剂量的环磷酰胺或替代它的其他免疫抑制剂和骨髓抑制剂。
A 10-year-old boy with Shwachman-Diamond syndrome and severe bone marrow failure was treated with highdose cyclophosphamide, busulfan, and antithymocyte globulin followed by an infusion of human leukocyte antigen-identical, mixed lymphocyte culture (MLC) nonreactive sibling bone marrow. He developed cardiac arrythmias and intractable hypotension and died on day 23 posttransplant. Autopsy findings were consistent with cyclophosphamide-induced pancarditis. The bone marrow showed signs of early engraftment. Allogeneic bone marrow transplantation may be a treatment alternative for Shwachman-Diamond syndrome with severe bone marrow failure. However, fatal posttransplant pancarditis due to doses of cyclophosphamide not usually associated with cardiac death may be an unanticipated problem. Further trials of bone marrow transplantation as therapy for this syndrome may be warranted, perhaps using lower doses of cyclophosphamide or substituting for it other immunosuppressive and myelosuppressive agents.