广泛型慢性移植物抗宿主病重叠综合征1例暨文献复习  

Overlap syndrome:a case report of extensive chronic graft-versus-host disease and literature review

作　　者：周燕霞 罗升惠 覃蔚璇 叶红坚[1,2] 王莺 陈文芳[1,2,3] 李志坚[1,2] 陈崴[1,2] 王欣[1,2] Zhou Yanxia;Luo Shenghui;Qin Weixuan;Ye Hongjian;Wang Ying;Chen Wenfang;Li Zhijian;Chen Wei;Wang Xin(Department of Nephrology,the First Affiliated Hospital,Sun Yat-sen University,Guangzhou 510080,China;Guangdong Provincial Key Laboratory of Nephrology,Guangzhou 510080,China;Department of Pathology,the First Affiliated Hospital,Sun Yat-sen University,Guangzhou 510080,China)

机构地区：[1]中山大学附属第一医院肾内科,广州510080 [2]国家卫生健康委员会肾脏病临床研究重点实验室(中山大学)、广东省肾脏病重点实验室,广州510080 [3]中山大学附属第一医院病理科,广州510080

出　　处：《中华肾脏病杂志》2023年第11期851-857,共7页Chinese Journal of Nephrology

基　　金：广东省肾脏病重点实验室(2020B1212060028);国家卫生健康委员会肾脏病临床研究重点实验室(中山大学)。

摘　　要：该文报告1例青年男性患者,同种异基因造血干细胞移植后出现移植物抗宿主病(graft versus host disease,GVHD)多脏器受累,临床表现多样,急慢性病程重叠发生:急性GVHD表现以高胆红素血症伴或不伴转氨酶升高、血性水样便为主;慢性GVHD以广泛皮肤色素脱失、口腔黏膜溃疡、病甲等表现突出,还有膜性肾病、多浆膜腔积液及肺限制性通气功能不全等慢性GVHD表现。GVHD诊断主要依靠病史和临床表现,鉴别排查感染、药物及肿瘤等因素影响,易漏诊误诊,需多学科诊治。文献回顾提示造血干细胞移植后男性受者女性供者、外周血造血干细胞移植者较骨髓移植者慢性GVHD发生风险高,但移植物抗白血病效应存在。目前糖皮质激素联合或不联合钙调磷酸酶抑制剂是GVHD的一线治疗方案,总体患者预后不佳。The paper reported a case of a young male patient,with graft-versus-host disease(GVHD)multi-organ involvement lesions after allo-hematopoietic stem cell transplantation.The patient had diverse clinical manifestations,and overlapping acute and chronic disease processes.Acute GVHD were mainly hyperbilirubinemia,with or without elevated transaminase,bloody watery stools;chronic GVHD were highlighted by extensive skin depigmentation,oral mucosal ulcer,sick nails,etc.,and chronic signs,such as membranous nephropathy,polyserositis and pulmonary restrictive ventilatory insufficiency.The diagnosis of chronic GVHD mainly relies on medical history combined with clinical manifestations,and it's needed to exclude infections,drugs and tumors.Besides,the rate of missed diagnosis and misdiagnose is high,and it requires multidisciplinary diagnosis and treatment.Combined with the literature review,it indicates that there is a greater risk of GVHD in the male recipient with female donor,and peripheral blood stem cell transplant patients have a higher incidence than bone marrow transplant patients after hematopoietic stem cell transplantation,but the effect of the graft-versus-leukemia exists.Currently,glucocorticoids therapy with or without calcineurin inhibitors are the first-line treatment for GVHD,but the overall prognosis is poor.

关 键 词：移植物抗宿主病 肾小球肾炎 膜性 移植物白血病反应 重叠综合征 浆膜腔积液 

分 类 号：R457.7[医药卫生—治疗学]