Many faces of graft-versus-host disease

Allogeneic haematopoietic stem cell transplantation is increasingly used in the treatment of malignant and non-malignant disorders. Despite ongoing advances in the field, morbidity and mortality related to graft- versus -host disease remains a major barrier to its application. Graft- versus -host disease is a difficult-to-diagnose disease. Dermatologists are involved due to its diverse cutaneous expression. In order to appropriately diagnose, classify and treat this complex disease, knowledge of its expanding cutaneous expression is required. This review provides a synopsis of the clinical manifestations of acute, lichenoid and sclerodermatous phases of graft- versus -host disease with a look at the current evidence surrounding its differential diagnosis.     

具有白癜风样表现的慢性移植物抗宿主病1例

患者男,21岁。口腔溃疡、全身发疹伴眉毛、睫毛变白2周。6个月前患者因"急性淋巴细胞白血病"行异体造血干细胞移植。查体示:躯干部弥漫性分布暗红斑,伴脱屑和苔藓样改变,口腔、舌黏膜白斑、糜烂出血,双侧眉毛和眼睫毛部分呈白色。诊断:慢性移植物抗宿主病。系国内首例局限于眉毛和睫毛的具有白癜风样表现的慢性移植物抗宿主病。     

银屑病患者骨髓CD34+细胞体外定向分化的T细胞活性研究

目的 研究有家族发病倾向的银屑病患者骨髓CD34⁺细胞体外定向发育的T细胞活性.方法 免疫磁珠法分离家族史阳性银屑病患者及正常对照骨髓CD34⁺细胞,在骨髓基质细胞条件培养液构建的微环境下,在胸腺基质细胞的支持下,使其在体外向T细胞定向分化,免疫磁珠法收集CD3⁺T细胞,流式细胞仪检测CD4⁺CD8^-细胞及CD4^-CD8⁺细胞比例.分别采用MTT法及ELISA法检测自然增殖组及链球菌超抗原刺激后T细胞增殖活性及分泌细胞因子水平.结果 ①经骨髓CD34⁺细胞定向分化并扩增的CD3⁺T细胞中可检测到CD4⁺CD8^-、CD4^-CD8⁺T细胞,且银屑病患者组与正常对照组CD4⁺CD8^-及CD4^-CD8⁺T细胞比例无显著差异.②银屑病患者骨髓CD34⁺细胞定向分化的T细胞自然增殖组及链球菌超抗原刺激组增殖活性均显著高于正常人对照组.③银屑病患者T细胞自然增殖组培养上清白介素4、白介素8及干扰素γ与正常对照组差异无统计学意义,链球菌超抗原刺激后白介素4表达水平无显著改变,而白介素8及干扰素γ水平却显著高于正常人.结论 有家族发病倾向的银屑病患者外周血T细胞活性异常可能与骨髓造血细胞相关.     

Cutaneous gamma‐delta T‐cell lymphoma with central nervous system involvement: report of a rarity with review of literature

Primary cutaneous gamma‐delta (γδ) T‐cell lymphoma is an extremely rare and aggressive variant of cutaneous lymphoma. Central nervous system (CNS) involvement, a rare finding, and hemophagocytic syndrome are two complications that are commonly fatal. We describe a 58‐year‐old patient presenting with skin plaque who subsequently developed subcutaneous nodules diagnosed as cutaneous T‐cell lymphoma (CTCL), clinically resembling ‘mycosis fungoides’. The patient was treated with repeat topical radiation therapies but had frequent relapsed disease. Approximately 4.5 years after, the patient presented with third and sixth cranial nerve palsies and was found to have CNS involvement by lymphoma per positron emission tomography—computed tomography (PET/CT) and a biopsy of foramen magnum. Phenotypically, the tumor cells were CD3(+)/CD4(?)/CD8(?)/CD7(+)/CD5(?)/CD30(?)/TCRαβ(?)/TCRγδ(+). Despite aggressive strategies taken, the patient expired 3 months after the diagnosis of the CNS lesion. A retrospective investigation proved the original CTCL to be γδ T‐cell in origin, confirming an indolent cutaneous γδ T‐cell lymphoma with eventual CNS manifestation. We present this case to draw attention to the entity, which can occasionally present with misleading histopathologic and clinical features. In addition, we provide a review of the literature to summarize clinical and pathologic features of the reported similar cases.