硬皮病样皮肤移植物抗宿主病24例临床特征分析  

Clinical features of 24 cases of scleroderma-like cutaneous graft-versus-host disease

作　　者：于聪[1] 周城[1] 张建中[1] Yu Cong;Zhou Cheng;Zhang Jianzhong(Department of Dermatology,Peking University People's Hospital,Beijing 100044,China)

机构地区：[1]北京大学人民医院皮肤科,北京100044

出　　处：《中华皮肤科杂志》2022年第2期123-128,共6页Chinese Journal of Dermatology

基　　金：国家自然科学基金(82073459)。

摘　　要：目的分析异基因造血干细胞移植后硬皮病样皮肤移植物抗宿主病(GVHD)的临床特征和危险因素。方法回顾2014-2019年间就诊于北京大学人民医院皮肤科硬皮病样皮肤GVHD患者24例的临床资料,分析临床特征、治疗、预后及发展为硬皮病样皮肤GVHD的可能因素。结果24例硬皮病样皮肤GVHD患者中男11例,女13例,年龄(33±12)岁,HLA全相合20例,半相合4例,发病时间[M(Q_(1),Q_(3))]为移植后18.5(8.0,30.9)个月。19例发病前已停用预防排异药或药物处于小剂量。15例表现为泛发性硬皮病样,1例为线状硬皮病样,5例硬斑病样,3例筋膜炎样。泛发性硬皮病样皮肤GVHD无雷诺征。13例伴有其他系统排异,8例合并关节活动受限,1例合并慢性皮肤溃疡并继发皮肤鳞状细胞癌。所有患者均给予系统糖皮质激素或联合免疫抑制剂,11例外用糖皮质激素。对11例患者进行集中随访,其中明显好转3例,好转4例,加重2例,死亡2例。以同时段就诊的223例非硬皮病样皮肤GVHD患者为对照,硬皮病样皮肤GVHD组HLA全相合患者比例(20/24,83.3%)高于非硬皮病样皮肤GVHD患者(47/223,21.1%),两组比较,P<0.001。结论硬皮病样皮肤GVHD平均发病时间较晚,可模仿所有4种自发性硬皮病的表现,HLA全相合移植、过早停用或预防排异药减量过快可能是发展至硬皮病样皮肤GVHD的危险因素。Objective To investigate clinical features of and risk factors for scleroderma-like cutaneous graft-versus-host disease(GVHD)after allogeneic hematopoietic stem cell transplantation.Methods Clinical data were collected from 24 patients with scleroderma-like cutaneous GVHD in Department of Dermatology,Peking University People's Hospital from 2014 to 2019.Clinical features,treatment,prognosis,and possible factors influencing the development of scleroderma-like cutaneous GVHD were analyzed retrospectively.Results Among the 24 patients,11 were males,and 13 were females,aged 33±12 years;20 were human leukocyte antigen(HLA)-identical recipients,4 were HLA-haploidentical recipients;GVHD occurred 18.5(8.0,30.9)months after transplantation.Nineteen patients had discontinued anti-rejection therapy or received low-dose anti-rejection drugs before the onset of GVHD.Fifteen patients presented with generalized scleroderma-like lesions,1 with linear scleroderma-like lesions,5 with morphea-like lesions,and 3 with fasciitis-like lesions.None of the 15 patients with generalized scleroderma-like GVHD had Raynaud syndrome.Thirteen patients were accompanied by graft rejection in other systems,8 had joint mobility limitations,and 1 developed cutaneous squamous cell carcinoma secondary to chronic skin ulcers.All patients were treated with systemic glucocorticoids and immunosuppressive agents,and 11 also with topical glucocorticoids.An intensive follow-up was carried out in 11 patients,of whom 3 achieved marked improvement,4 achieved improvement,2 experienced exacerbation,and 2 died.A total of 223 patients with non-sclerodermatous cutaneous GVHD admitting during the same period served as controls,and the proportion of HLA-identical patients was significantly higher in the scleroderma-like cutaneous GVHD group(20/24,83.3%)than in the non-sclerodermatous cutaneous GVHD group(47/223,21.1%;P<0.001).Conclusions Scleroderma-like cutaneous GVHD commonly occurs late,and can mimic clinical manifestations of all 4 types of spontaneous scleroderm

关 键 词：移植物抗宿主病 造血干细胞移植 硬皮病样 危险因素 

分 类 号：R593.25[医药卫生—内科学]