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实体器官移植后原发性皮肤T细胞淋巴瘤的特征分析

Characterization of primary cutaneous T-cell lymphoma following solid organ transplantation.

作者信息

Shimshak Serena, Dai Christina, Comfere Nneka, Sokumbi Olayemi

机构信息

Alix School of Medicine, Mayo Clinic, Jacksonville, FL, USA.

Department of Dermatology, Mayo Clinic, Jacksonville, FL, USA.

出版信息

Int J Dermatol. 2023 Apr;62(4):494-500. doi: 10.1111/ijd.16300. Epub 2022 Jun 10.

Abstract

BACKGROUND

Immunosuppression following solid organ transplantation is a known risk factor for the development of posttransplant lymphoproliferative disorders (PTLD). Primary cutaneous T-cell lymphoma (CTCL) occurring in the posttransplant setting is rare, which has made comprehensive understanding of this disease challenging. This study aims to further characterize the spectrum of clinicopathologic features of CTCL in solid organ transplant recipients (SOTR).

METHODS

A retrospective chart review was performed for SOTR who were diagnosed with CTCL at a multi-site academic medical center from January 1, 1998, to December 31, 2013. Eight patients fulfilled the inclusion criteria and were included in this study. Data collected included patient demographics, transplanted organ, the time between transplant and CTCL diagnosis, clinical presentation and rash morphology, a histological subtype of CTCL, immunosuppression regimens, and patient status. Twelve diagnostic skin biopsies for five patients were examined and reviewed by a board-certified dermatopathologist.

RESULTS

Six (75%) out of the eight patients were men, two (25%) were women, and the median age was 53 years. The median time from the date of transplant to the diagnosis of CTCL was 8.2 years. Transplanted organs included the liver (4), kidney (3), and heart (1). Clinical presentation varied from papulonodules, comedone-like lesions, intense pruritis, and scaly erythematous eruptions. The most common histologic presentation was folliculotropic mycosis fungoides (FMF) (7/12). Epstein-Barr virus-in situ hybridization (EBV-ISH) was negative in all specimens.

CONCLUSIONS

We emphasize the rarity of CTCL among SOTR. Although rare in the general population, the FMF subtype appears to be disproportionately seen in SOTR compared with other CTCL.

摘要

背景

实体器官移植后的免疫抑制是移植后淋巴组织增生性疾病(PTLD)发生的已知危险因素。移植后发生的原发性皮肤T细胞淋巴瘤(CTCL)很罕见,这使得全面了解这种疾病具有挑战性。本研究旨在进一步描述实体器官移植受者(SOTR)中CTCL的临床病理特征谱。

方法

对1998年1月1日至2013年12月31日在一家多中心学术医疗中心被诊断为CTCL的SOTR进行回顾性病历审查。8例患者符合纳入标准并被纳入本研究。收集的数据包括患者人口统计学资料、移植器官、移植与CTCL诊断之间的时间、临床表现和皮疹形态、CTCL的组织学亚型、免疫抑制方案以及患者状态。由一位获得委员会认证的皮肤病理学家对5例患者的12份诊断性皮肤活检标本进行检查和复查。

结果

8例患者中有6例(75%)为男性,2例(25%)为女性,中位年龄为53岁。从移植日期到CTCL诊断的中位时间为8.2年。移植器官包括肝脏(4例)、肾脏(3例)和心脏(1例)。临床表现多样,包括丘疹结节、粉刺样损害、剧烈瘙痒和鳞屑性红斑疹。最常见的组织学表现是亲毛囊性蕈样肉芽肿(FMF)(7/12)。所有标本的爱泼斯坦-巴尔病毒原位杂交(EBV-ISH)均为阴性。

结论

我们强调CTCL在SOTR中很罕见。尽管在普通人群中罕见,但与其他CTCL相比,FMF亚型在SOTR中似乎更为常见。

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