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异基因骨髓移植后阻塞性气道疾病免疫抑制治疗的长期随访

Long-term follow-up of immunosuppressive treatment for obstructive airways disease after allogeneic bone marrow transplantation.

作者信息

Sánchez J, Torres A, Serrano J, Román J, Martín C, Pérula L, Martínez F, Gómez P

机构信息

Haematology Service, University Hospital Reina Sofia, Córdoba, Spain.

出版信息

Bone Marrow Transplant. 1997 Sep;20(5):403-8. doi: 10.1038/sj.bmt.1700894.

DOI:10.1038/sj.bmt.1700894
PMID:9339757
Abstract

To describe clinical outcome with first line immunosuppression therapy for obstructive airways disease (OAD) after allogeneic BMT, we have retrospectively examined 20 long-term survivors affected by OAD. All patients had normal pulmonary function test (PFTs) before BMT. OAD was defined as FEV1 less than 80%, FER less than 80%, maximum midexpiratory flow rate of 50% vital capacity (MMFR) less than 65%, or residual volume greater than 120. Prednisone (n = 4), CsA (n = 8) and azathioprine (n = 8) have been used as first-line immunosuppression agents. Mean follow-up was 65 months (range 15-142). We identified three categories of patients according to response to treatment: complete (n = 6, 30%), partial (n = 6, 30%) or no response (n = 8, 40%). Age, FEV1, time of onset after BMT, Karnofsky index or immunosuppression modality do not seem to be related to subsequent response. However, patients with low values of MMFR and high values of RV at the beginning of therapy are likely to show poor response. In the complete response group, normalisation of PFTs is achieved within the first months of treatment (median 6 months ranging from 3 to 9 months), suggesting that prolonged therapy is not advantageous and could increase morbidity and mortality if there are no other signs of CGVHD.

摘要

为描述异基因骨髓移植后阻塞性气道疾病(OAD)一线免疫抑制治疗的临床结果,我们回顾性研究了20例受OAD影响的长期存活者。所有患者在骨髓移植前肺功能测试(PFTs)均正常。OAD定义为第一秒用力呼气容积(FEV1)低于80%、用力呼气率(FER)低于80%、50%肺活量时的最大呼气中期流速(MMFR)低于65%或残气量大于120。泼尼松(n = 4)、环孢素(CsA,n = 8)和硫唑嘌呤(n = 8)已被用作一线免疫抑制剂。平均随访时间为65个月(范围15 - 142个月)。我们根据治疗反应将患者分为三类:完全缓解(n = 6,30%)、部分缓解(n = 6,30%)或无反应(n = 8,40%)。年龄、FEV1、骨髓移植后发病时间、卡诺夫斯基指数或免疫抑制方式似乎与后续反应无关。然而,治疗开始时MMFR值低和RV值高的患者可能反应较差。在完全缓解组中,治疗的头几个月内(中位数6个月,范围3至9个月)PFTs恢复正常,这表明如果没有慢性移植物抗宿主病(CGVHD)的其他迹象,延长治疗并无益处,反而可能增加发病率和死亡率。

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