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造血干细胞移植后移植物抗宿主病患者肺嗜酸性粒细胞增多症的罕见并发症和诊断挑战。

The Rare Complication and Diagnostic Challenges of Pulmonary Eosinophilia in Graft versus Host Disease Patients after Hematopoietic Stem Cell Transplantation.

机构信息

Department of Medicine, University of Minnesota, 131 VCRC, 401 E River Pkwy, Minneapolis, 55455, MN, USA.

Division of Pulmonary, Allergy, Critical Care and Sleep Medicine, University of Minnesota, 420 Delaware SE, MMC 276, Minneapolis, 55455, MN, USA.

出版信息

Lung. 2017 Dec;195(6):805-811. doi: 10.1007/s00408-017-0060-z. Epub 2017 Oct 20.

Abstract

PURPOSE

Chronic graft versus host disease (cGvHD) is a common complication of hematopoietic stem cell transplantation (HSCT). Eosinophilic lung disease is a rare poorly understood complication in HSCT patients with cGvHD. These patients present similarly to those with Acute Eosinophilic Pneumonia (AEP). The purpose of this study is to better elucidate the presentation and potential treatment of this phenomenon.

METHODS

We reviewed over 170 bronchoscopies in post-HSCT patients with respiratory symptoms. Of these, four patients, whose course was complicated by cGvHD, presented with respiratory symptoms, diffuse ground-glass opacities (GGO) on chest computerized tomography (CT), bronchoalveolar lavage (BAL) eosinophilia, and no evidence of infection. The clinical course of these patients was reviewed.

RESULTS

Despite clinical presentation similar to AEP, not all patients had > 25% eosinophils on BAL, one criterion for AEP, however all improved with steroids. Steroid initiation was often delayed in favor of empiric antibiotics despite negative infectious workup. Several patients had recurrent episodes. Regarding possible associations, we examined but found no link between particular demographics, reason for HSCT, chemotherapy, immunosuppressants, or peripheral eosinophil count and pulmonary eosinophilia in these patients. GGO present on initial CT imaging became chronic in several of these patients.

CONCLUSION

We propose that in post-HSCT patients with GvHD presenting with respiratory symptoms, GGO on CT, BAL eosinophilia of > 10%, and negative respiratory cultures, an autoimmune eosinophilic process may be occurring. Earlier recognition and initiation of corticosteroids in these patients may improve their outcomes as an autoimmune diagnosis was often delayed in favor of antibiotics.

摘要

目的

慢性移植物抗宿主病(cGvHD)是造血干细胞移植(HSCT)的常见并发症。嗜酸性肺疾病是 HSCT 伴 cGvHD 患者中一种罕见且了解甚少的并发症。这些患者的表现与急性嗜酸性肺炎(AEP)患者相似。本研究旨在更好地阐明这种现象的表现和潜在治疗方法。

方法

我们回顾了 170 多例 HSCT 后出现呼吸系统症状患者的支气管镜检查。其中,4 例患者因 cGvHD 病程复杂,出现呼吸系统症状、弥漫性磨玻璃影(GGO)、支气管肺泡灌洗(BAL)嗜酸性粒细胞增多,且无感染证据。回顾了这些患者的临床病程。

结果

尽管临床表现类似于 AEP,但并非所有患者的 BAL 嗜酸性粒细胞计数均>25%,AEP 的一个标准,但所有患者均对类固醇治疗有效。尽管进行了阴性感染性检查,但类固醇的起始通常被延迟,转而采用经验性抗生素治疗。一些患者反复发作。关于可能的关联,我们进行了检查,但未发现这些患者的特定人口统计学特征、HSCT 原因、化疗、免疫抑制剂或外周嗜酸性粒细胞计数与肺部嗜酸性粒细胞增多之间存在关联。这些患者的初始 CT 影像学上的 GGO 变为慢性。

结论

我们提出,在伴有 GvHD 的 HSCT 后患者出现呼吸系统症状、CT 上的 GGO、BAL 嗜酸性粒细胞计数>10%且呼吸道培养阴性时,可能发生自身免疫性嗜酸性粒细胞增多症。在这些患者中,早期识别和开始使用皮质类固醇可能会改善他们的预后,因为自身免疫性诊断往往被延迟,而有利于使用抗生素。

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