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一名患有X连锁淋巴增殖综合征和急性重症传染性单核细胞增多症的男孩成功接受骨髓移植。

Successful bone marrow transplantation in a boy with X-linked lymphoproliferative syndrome and acute severe infectious mononucleosis.

作者信息

Pracher E, Panzer-Grümayer E R, Zoubek A, Peters C, Gadner H

机构信息

St. Anna Children's Hospital, Vienna, Austria.

出版信息

Bone Marrow Transplant. 1994 May;13(5):655-8.

PMID:8054918
Abstract

We report a 5.9-year-old boy with X-linked lymphoproliferative syndrome (XLP) who presented with acute severe infectious mononucleosis. Clinical symptoms rapidly improved after chemotherapy with etoposide. Allogeneic bone marrow transplantation (BMT) was performed after conditioning with etoposide, busulfan and cyclophosphamide. After successful hematopoietic recovery we were able to demonstrate seroconversion from an impaired antibody response to Epstein-Barr virus (EBV) to a normal antibody-producing state in an immunocompetent child. The only post-transplant complication was mild acute graft-versus-host disease (GVHD). Three years after BMT, the boy is healthy and shows no signs of immunodeficiency. This is the first report on successful allogeneic BMT in the severe course of acute infectious mononucleosis in a patient with XLP. We speculate that the application of etoposide contributed to the positive outcome in this patient.

摘要

我们报告了一名5.9岁患有X连锁淋巴增生综合征(XLP)的男孩,他表现为急性重症传染性单核细胞增多症。使用依托泊苷进行化疗后,临床症状迅速改善。在用依托泊苷、白消安和环磷酰胺进行预处理后,进行了异基因骨髓移植(BMT)。在造血功能成功恢复后,我们能够证明在一名免疫功能正常的儿童中,从对EB病毒(EBV)抗体反应受损转变为正常的抗体产生状态的血清学转换。唯一的移植后并发症是轻度急性移植物抗宿主病(GVHD)。BMT三年后,该男孩身体健康,没有免疫缺陷的迹象。这是关于XLP患者在急性传染性单核细胞增多症严重病程中成功进行异基因BMT的首例报告。我们推测依托泊苷的应用促成了该患者的良好预后。

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