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造血干细胞移植治疗 Wiskott-Aldrich 综合征后行肺移植。

Lung transplantation after haematopoietic stem cell transplantation for Wiskott-Aldrich syndrome.

机构信息

Department of Thoracic Surgery, Kyoto University, Sakyo-ku, Kyoto, Japan.

出版信息

Eur J Cardiothorac Surg. 2018 Jun 1;53(6):1286-1287. doi: 10.1093/ejcts/ezx461.

DOI:10.1093/ejcts/ezx461
PMID:29309544
Abstract

The authors report the first case involving a patient with Wiskott-Aldrich syndrome who underwent single living-donor lobar lung transplantation after haematopoietic stem cell transplantation. Haematopoietic stem cell transplantation was performed at 1 year of age; however, he developed severe pulmonary complications. Although lung transplantation is generally contraindicated in patients with immunodeficiency disease, the patient was able to undergo living-donor lobar lung transplantation because his immunodeficiency and thrombocytopenia were well controlled as a result of haematopoietic stem cell transplantation. Currently, the patient is doing well and is free from oxygen supplementation.

摘要

作者报告了首例接受造血干细胞移植后行单肺叶供体肺移植的 Wiskott-Aldrich 综合征患者。患者于 1 岁时接受造血干细胞移植,但随后发生严重肺部并发症。尽管肺移植一般禁忌用于免疫缺陷疾病患者,但由于造血干细胞移植可良好控制患者的免疫缺陷和血小板减少症,该患者仍可接受肺叶供体肺移植。目前,患者情况良好,无需吸氧。

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