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实体器官移植受者原发性皮肤移植后淋巴组织增生性疾病:一项多中心欧洲病例系列研究。

Primary cutaneous posttransplant lymphoproliferative disorders in solid organ transplant recipients: a multicenter European case series.

机构信息

Department of Dermatology, Başkent University Faculty of Medicine, Ankara, Turkey.

出版信息

Am J Transplant. 2013 Aug;13(8):2146-53. doi: 10.1111/ajt.12281. Epub 2013 May 29.

Abstract

Primary cutaneous posttransplant lymphoproliferative disorders (PTLD) are rare. This retrospective, multicenter study of 35 cases aimed to better describe this entity. Cases were (re)-classified according to the WHO-EORTC or the WHO 2008 classifications of lymphomas. Median interval between first transplantation and diagnosis was 85 months. Fifty-seven percent of patients had a kidney transplant. Twenty-four cases (68.6%) were classified as primary cutaneous T cell lymphoma (CTCL) and 11 (31.4%) as primary cutaneous B cell PTLD. Mycosis fungoides (MF) was the most common (50%) CTCL subtype. Ten (90.9%) cutaneous B cell PTLD cases were classified as EBV-associated B cell lymphoproliferations (including one plasmablastic lymphoma and one lymphomatoid granulomatosis) and one as diffuse large B cell lymphoma, other, that was EBV-negative. Sixteen (45.7%) patients died after a median follow-up of 19.5 months (11 [68.8%] with CTCL [6 of whom had CD30(+) lymphoproliferative disorders (LPD)] and 5 [31.2%] with cutaneous B cell PTLD. Median survival times for all patients, CTCL and cutaneous B cell PTLD subgroups were 93, 93, and 112 months, respectively. Survival rates for MF were higher than those for CD30(+) LPD. The spectrum of primary CTCL in organ transplant recipients (OTR) is similar to that in the general population. The prognosis of posttransplant primary cutaneous CD30(+) LPD is worse than posttransplant MF and than its counterpart in the immunocompetent population. EBV-associated cutaneous B cell LPD predominates in OTR.

摘要

原发性皮肤移植后淋巴组织增生性疾病(PTLD)较为罕见。本回顾性多中心研究纳入了 35 例患者,旨在更好地描述该疾病。病例根据世界卫生组织-欧洲癌症研究与治疗组织(WHO-EORTC)或世界卫生组织 2008 年淋巴瘤分类进行(再)分类。首次移植与诊断之间的中位间隔为 85 个月。57%的患者接受了肾移植。24 例(68.6%)被分类为原发性皮肤 T 细胞淋巴瘤(CTCL),11 例(31.4%)为原发性皮肤 B 细胞 PTLD。蕈样真菌病(MF)是最常见的(50%)CTCL 亚型。10 例(90.9%)皮肤 B 细胞 PTLD 病例被分类为 EBV 相关 B 细胞淋巴增生(包括 1 例浆母细胞淋巴瘤和 1 例淋巴组织细胞性血管炎),1 例为弥漫性大 B 细胞淋巴瘤,其他为 EBV 阴性。16 例(45.7%)患者在中位随访 19.5 个月后死亡(11 例 CTCL [其中 6 例为 CD30(+)淋巴增生性疾病]和 5 例皮肤 B 细胞 PTLD)。所有患者、CTCL 和皮肤 B 细胞 PTLD 亚组的中位生存时间分别为 93、93 和 112 个月。MF 的生存率高于 CD30(+) LPD。移植后原发性 CTCL 在器官移植受者(OTR)中的谱与一般人群相似。移植后原发性皮肤 CD30(+) LPD 的预后比移植后 MF 和免疫功能正常人群的 CD30(+) LPD 更差。EBV 相关的皮肤 B 细胞 LPD 在 OTR 中更为常见。

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