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异基因骨髓移植后与微血管病相关的弥漫性肺泡出血

Diffuse alveolar haemorrhage associated with microangiopathy after allogeneic bone marrow transplantation.

作者信息

Srivastava A, Gottlieb D, Bradstock K F

机构信息

Haematology Department, Westmead Hospital, NSW, Australia.

出版信息

Bone Marrow Transplant. 1995 Jun;15(6):863-7.

PMID:7581082
Abstract

Microangiopathic disease and diffuse alveolar haemorrhage (DAH) are uncommon serious complications of bone marrow transplantation (BMT), but an association between these two conditions has not been previously recognised. We report 4 patients in whom these two complications occurred after allogeneic BMT for haematological malignancy. The patients were 16-39 years of age, and received transplants for acute myeloid leukemia, chronic myeloid leukemia and non-Hodgkin's lymphoma (n = 2). Donors were HLA-identical siblings (n = 3), and a matched unrelated volunteer. The patient with AML was receiving a second transplant for relapse 3 years after her first BMT, and was prepared with busulphan and melphalan; other patients received total body irradiation and cyclophosphamide. Microangiopathy occurred 20-48 days after BMT, and was associated with renal impairment in all cases, and mental confusion in 3. Cyclosporin levels were in the toxic range in 2 cases. DAH occurred 18-55 days after BMT, in 3 cases 2-7 days after the onset of microangiopathy, but preceding it by 14 days in the other case. Patients were treated with fresh frozen plasma, plasma exchange, supplemental oxygen and ventilation in 2 cases. Two patients died of progressive respiratory failure, while 2 patients recovered with evidence of continuing microangiopathic disease, and died of myocardial infarction or fungal infection. We report an association between microangiopathic disease and DAH in these BMT patients, and suggest that damage to the pulmonary vascular endothelium may be the common pathophysiological event, although no specific causative factor could be identified.

摘要

微血管病和弥漫性肺泡出血(DAH)是骨髓移植(BMT)罕见的严重并发症,但此前尚未认识到这两种情况之间的关联。我们报告了4例在异基因BMT治疗血液系统恶性肿瘤后发生这两种并发症的患者。患者年龄在16 - 39岁之间,接受移植治疗的疾病包括急性髓系白血病、慢性髓系白血病和非霍奇金淋巴瘤(2例)。供者为HLA匹配的同胞(3例)和1例匹配的无关志愿者。患有急性髓系白血病的患者在首次BMT后3年因复发接受第二次移植,预处理方案为白消安和马法兰;其他患者接受全身照射和环磷酰胺。微血管病发生在BMT后20 - 48天,所有病例均伴有肾功能损害,3例伴有精神错乱。2例患者的环孢素水平处于中毒范围。DAH发生在BMT后18 - 55天,3例在微血管病发作后2 - 7天出现,另1例则在微血管病发作前14天出现。患者接受了新鲜冷冻血浆、血浆置换、补充氧气治疗,2例患者还接受了通气治疗。2例患者死于进行性呼吸衰竭,2例患者康复,但仍有微血管病证据,最终死于心肌梗死或真菌感染。我们报告了这些BMT患者中微血管病与DAH之间的关联,并提示肺血管内皮损伤可能是共同的病理生理事件,尽管未能确定具体的致病因素。

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