Bristol Cup Posters
Bristol Cup Posters
复制标题
布里斯托尔杯海报
DOI：
10.1111/bjd.16406
复制
发表时间：
2018
影响因子：
10.3
通讯作者：
A. Y. Finlay
中科院分区：
文献类型：
--
作者：
F. M. Hajjaj;M. Salek;M. Basra;A. Y. Finlay
一种罕见的起泡原因：侵袭性大疱性皮肤T细胞淋巴瘤N.Watson，S.Fatah，C.Bacon，J.Frew和S.Weatherhead皇家维多利亚医院，英国纽卡斯尔，泰恩河畔，英国，詹姆斯·库克大学医院，英国米德尔斯堡，北方癌症护理中心，英国纽卡斯尔，泰恩河畔。18个月前，他有多处广泛的红斑块和斑块，在社区中被视为“湿疹”。他的手/脚上可见紧张的水泡，其中一些重叠在现有的斑块上。他的前臂上有几个大肿瘤，有广泛的红斑块。皮肤活检显示弥漫性非典型淋巴组织渗入，影响真皮厚度并延伸至皮下脂肪。浸润物由中等大小的异型细胞、小淋巴细胞、巨噬细胞和大的核样淋巴细胞组成。最小的表皮亲和性和毛囊亲和性。非典型淋巴样细胞表达CD3和CD2，但不表达CD4、CD5、CD8、TCR-β、TCR-γ、CD30、CD56或细胞毒蛋白。EBV-EBER原位杂交均为阴性。Ki67增殖指数为80%。PCR克隆性表现为克隆性TCRG和TCRD基因重排。直接免疫荧光为阴性。CT扫描显示双侧腹股沟淋巴结10 mm。经过多学科的讨论，临床病理特征最符合侵袭性的大疱性真菌样肉芽肿(MF)，很可能是伽马/德尔塔T细胞系。R-CHOP化疗取得了初步的良好反应，但他的疾病在2周内复发。治疗改用脂质体阿霉素，皮损几乎完全消失。随后，他出现了眼睛疼痛、红斑，视力恶化。玻璃体活检证实淋巴瘤受累，但核磁共振未显示中枢神经系统疾病。全皮肤电子束治疗被送到整个皮肤表面(30格雷)；他的眼眶接受单独的放射治疗。随后的成像显示完全缓解，所以他进行了兄弟姐妹异基因干细胞移植，但复发并在2个月后死亡。进展期进行的皮肤活检显示出与早期活检相似的特征，但现在显示出细胞毒蛋白的表达。因此，我们重新探讨了伽马-德尔塔T细胞淋巴瘤(TCL)的鉴别诊断，尽管总体的临床病理图像被认为与大疱性MF最为一致。大疱性真菌样肉芽肿是一种极其罕见的MF，在侵袭性伽玛-三角洲TCL中没有水泡的报道。它出现在没有性取向的老年患者中。囊泡性皮损通常出现在疾病的典型斑块/斑块阶段数月至数年后，但很少是MF的主要表现。关于这种罕见表型的有限文献表明预后不良(Bowman PH，Hogan DJ，Sanusi ID)。真菌大疱性霉菌病：1例报告和文献回顾。J Am Acad Dermatol 2001；45：934-9)。
P001 A rare cause of blistering: aggressive bullous cutaneous T-cell lymphoma N. Watson, S. Fatah, C. Bacon, J. Frew and S. Weatherhead Royal Victoria Infirmary, Newcastle Upon Tyne, U.K., The James Cook University Hospital, Middlesbrough, U.K. and Northern Centre for Cancer Care, Newcastle Upon Tyne, U.K. A 58-year-old man presented with a 3-month history of acute onset tense blisters on his hands and feet without systemic symptoms or recent medication exposure. Eighteen months prior, he had multiple widespread erythematous patches and plaques which were treated as ‘eczema’ in the community. Tense bullae were visible on his hands/feet, some superimposed on existing plaques. There were several large tumours on his forearms, with widespread erythematous patches. Skin biopsy showed a diffuse atypical lymphoid infiltrate affecting the thickness of the dermis and extending into subcutaneous fat. The infiltrate was composed of medium-sized atypical cells with irregular hyperchromatic nuclei, admixed small lymphocytes and macrophages and large nucleolated lymphoid cells. Minimal epidermotropism and folliculotropism were observed. The atypical lymphoid cells expressed CD3 and CD2, but no CD4, CD5, CD8, TCR-beta, TCR-gamma, CD30, CD56 or cytotoxic proteins. They were negative for EBV-EBER in situ hybridization. The Ki67 proliferation index was 80%. PCRbased clonality showed clonal TCRG and TCRD gene rearrangements. Direct immunofluorescence was negative. CT scan showed 10 mm inguinal lymph nodes bilaterally. After multidisciplinary discussion, the clinicopathological picture was most consistent with an aggressive form of bullous mycosis fungoides (MF), probably of gamma/delta T-cell lineage. R-CHOP chemotherapy achieved an initial good response but his disease relapsed within 2 weeks. Treatment was switched to liposomal doxorubicin with near complete resolution of his lesions. Subsequently, he developed painful, erythematous eyes with deteriorating vision. Vitreous biopsy confirmed lymphomatous involvement but MRI showed no CNS disease. Total skin electron beam therapy was delivered to the entire skin surface (30 Gray); separate radiotherapy was given to his orbits. Subsequent imaging showed complete remission, so he proceeded to sibling allogenic stem cell transplantation but relapsed and died 2 months later. Skin biopsy performed at progression showed similar features to earlier biopsies but now demonstrated cytotoxic protein expression. The differential diagnosis of gamma-delta T-cell lymphoma (TCL) was therefore re-visited, although the overall clinicopathological picture was considered most consistent with bullous MF. Bullous mycosis fungoides is an extremely rare form of MF, and blisters are not reported in aggressive gamma-delta TCL. It presents in older patients with no sex predilection. Vesiculobullous lesions usually appear months-years after classical patches/ plaques stage of the disease but can rarely be the primary manifestation of MF. The limited literature on this rare phenotype suggests a poor prognosis (Bowman PH, Hogan DJ, Sanusi ID. Mycosis fungoides bullosa: report of a case and review of the literature. J Am Acad Dermatol 2001; 45: 934–9).