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指甲营养不良、水肿和嗜酸性粒细胞增多:儿童严重慢性皮肤移植物抗宿主病的先兆。

Nail dystrophy, edema, and eosinophilia: harbingers of severe chronic GVHD of the skin in children.

作者信息

Huang J T, Duncan C N, Boyer D, Khosravi H, Lehmann L E, Saavedra A

机构信息

Dermatology Program, Boston Children's Hospital, Boston, MA, USA.

Department of Pediatric Oncology, Dana-Farber Cancer Institute, Boston, MA, USA.

出版信息

Bone Marrow Transplant. 2014 Dec;49(12):1521-7. doi: 10.1038/bmt.2014.194. Epub 2014 Sep 22.

Abstract

The prognostic value of adnexal findings in chronic GVHD (cGVHD) has not been investigated in children. Dermatologic examinations were performed in a severe cohort of 11 children with skin cGVHD seen over a 2-year period. Findings were compared with 25 additional patients with skin cGVHD and 97 control patients. In 36 patients with skin cGVHD, nail dystrophy was present in 45% of patients, and was significantly associated with sclerotic disease and lung cGVHD. Pterygium inversum unguis (PIU) was associated with severe lung disease, with significantly lower % predicted FVC and FEV1 in those with PIU than those without. Forty-four percent of GVHD patients had preceding peripheral edema and 56% had preceding peripheral eosinophilia. Peripheral edema and eosinophilia were significantly associated with sclerotic cGVHD and persisted until the diagnosis of cGVHD in all patients. Comparison of data with control patients showed that incidence of nail dystrophy, incidence of peripheral edema and mean peak peripheral eosinophil count of patients with skin cGVHD was significantly higher than those without cGVHD. This study suggests that nail dystrophy, persistent peripheral edema and persistent peripheral eosinophilia are harbingers of severe cGVHD of the skin in children. The presence of PIU may be a harbinger of severe lung involvement.

摘要

附件检查结果在儿童慢性移植物抗宿主病(cGVHD)中的预后价值尚未得到研究。对在两年期间观察到的11名患有皮肤cGVHD的重症儿童队列进行了皮肤科检查。将检查结果与另外25名皮肤cGVHD患者和97名对照患者进行了比较。在36名皮肤cGVHD患者中,45%的患者存在甲营养不良,且与硬化性疾病和肺部cGVHD显著相关。反向翼状胬肉(PIU)与严重肺部疾病相关,有PIU的患者预计FVC和FEV1的百分比显著低于无PIU的患者。44%的GVHD患者先前有外周水肿,56%的患者先前有外周嗜酸性粒细胞增多。外周水肿和嗜酸性粒细胞增多与硬化性cGVHD显著相关,并且在所有患者中一直持续到cGVHD诊断时。与对照患者的数据比较显示,皮肤cGVHD患者的甲营养不良发生率、外周水肿发生率和外周嗜酸性粒细胞平均峰值计数显著高于无cGVHD的患者。这项研究表明,甲营养不良、持续性外周水肿和持续性外周嗜酸性粒细胞增多是儿童严重皮肤cGVHD的先兆。PIU的存在可能是严重肺部受累的先兆。

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