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一名肾移植患者出现溃疡性坏死丘疹和结节,诊断为迟发性爱泼斯坦-巴尔病毒阴性移植后淋巴细胞增生性疾病

Late-onset Epstein-Barr virus-negative post-transplant lymphoproliferative disorder presenting as ulcerated necrotic papules and nodules in a renal transplant patient.

作者信息

Tang Mark B Y, Tan Eugene S T, Tan Suat Hoon

机构信息

National Skin Centre, Singapore.

出版信息

Australas J Dermatol. 2008 May;49(2):100-2. doi: 10.1111/j.1440-0960.2008.00438.x.

Abstract

We report a case of B-cell post-transplant lymphoproliferative disorder in a 57-year-old female 19 years postrenal transplant patient who presented with multiple, progressive, painful ulcerated necrotic papules and nodules over the left leg. Histopathological examination showed diffuse infiltration of the dermis by large atypical B-lymphoid cells, with a negative in situ hybridization test for Epstein-Barr virus. Gastrointestinal involvement was evident by the presence of atypical lymphoid cells in the peritoneal fluid. She only had partial response to localized radiotherapy and intravenous rituximab and died 4 months later of septicaemia. Unusual features highlighted in this case include the very late onset of disease 19 years post-transplant, Epstein-Barr virus negativity and the aggressive course of disease that did not respond to the reduction of immunosuppression, localized electron beam therapy and intravenous rituximab.

摘要

我们报告了一例57岁女性肾移植术后19年发生的B细胞移植后淋巴细胞增殖性疾病,该患者左腿出现多个进行性疼痛性溃疡坏死丘疹和结节。组织病理学检查显示真皮内有大量非典型B淋巴细胞弥漫浸润,爱泼斯坦-巴尔病毒原位杂交检测为阴性。腹腔积液中存在非典型淋巴细胞表明有胃肠道受累。她对局部放疗和静脉注射利妥昔单抗仅部分缓解,4个月后死于败血症。该病例突出的不寻常特征包括移植后19年疾病很晚才发生、爱泼斯坦-巴尔病毒阴性以及疾病进展迅速,对免疫抑制减量、局部电子束治疗和静脉注射利妥昔单抗均无反应。

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