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皮肤移植后淋巴组织增生性疾病

Cutaneous Post-transplant Lymphoproliferative Disorder.

作者信息

Kanji Alpa, Ahmad Raida, Bala Laksha, Mallon Eleanor

机构信息

Dermatology, Imperial College Healthcare NHS Trust, London, GBR.

Pathology, Imperial College Healthcare NHS Trust, London, GBR.

出版信息

Cureus. 2024 Jul 6;16(7):e63951. doi: 10.7759/cureus.63951. eCollection 2024 Jul.

Abstract

Post-transplant lymphoproliferative disorders (PTLD) involve T- or B-cell proliferation in an immunosuppressed transplant recipient. It usually presents at extra-nodal sites and can affect several organs. Cutaneous manifestations of PTLD are relatively rare and can be very heterogeneous. We report a case of a 36-year-old male cardiac transplant recipient on long-term immunosuppression (ciclosporin, azathioprine, and prednisolone) who presented with a three-month history of a painless ulcer on the right lower leg. A skin biopsy showed a dermal atypical lymphoid infiltrate positive for PAX5, CD20 and MUM1 on immunohistochemistry and EBV with in-situ hybridisation and a 70% Ki-67 cell proliferation index. A whole body fluorodeoxyglucose (FDG) positron emission tomography (PET) scan showed increased tracer uptake corresponding to the site of the cutaneous ulcer, the anterior cortex of the right lower tibia, an area adjacent to the right superficial femoral artery and the right inguinal node. These findings were in keeping with monomorphic B-cell post-transplant lymphoproliferative disorder (PTLD) consistent with diffuse large B-cell lymphoma, non-germinal centre subtype. Cessation of azathioprine and treatment with an anti-CD20 antibody, rituximab, led to clinical resolution of the ulcer and a negative FDG-PET scan, with no disease recurrence to date. We present a rare case of monomorphic PTLD with cutaneous involvement, presenting with a solitary, painless ulcer, which was successfully treated with a reduction in immunosuppression and additional rituximab monotherapy, given the aggressive subtype. PTLD can arise many years post-transplant and is a serious, potentially life-threatening complication. Therefore, early recognition and prompt treatment are of paramount importance.

摘要

移植后淋巴细胞增生性疾病(PTLD)是指免疫抑制的移植受者体内T细胞或B细胞发生增殖。它通常出现在结外部位,可累及多个器官。PTLD的皮肤表现相对罕见,且可能非常多样化。我们报告一例36岁男性心脏移植受者,长期接受免疫抑制治疗(环孢素、硫唑嘌呤和泼尼松龙),右小腿出现一个无痛性溃疡,病程3个月。皮肤活检显示真皮非典型淋巴细胞浸润,免疫组化PAX5、CD20和MUM1阳性,原位杂交检测EBV阳性,Ki-67细胞增殖指数为70%。全身氟脱氧葡萄糖(FDG)正电子发射断层扫描(PET)显示,与皮肤溃疡部位、右胫骨前皮质、右股浅动脉相邻区域及右腹股沟淋巴结对应的示踪剂摄取增加。这些发现符合单形性B细胞移植后淋巴细胞增生性疾病(PTLD),与弥漫性大B细胞淋巴瘤非生发中心亚型一致。停用硫唑嘌呤并使用抗CD20抗体利妥昔单抗治疗后,溃疡临床消退,FDG-PET扫描转阴,至今无疾病复发。我们报告了一例罕见的单形性PTLD伴皮肤受累病例,表现为孤立性无痛性溃疡,鉴于其侵袭性亚型,通过减少免疫抑制和额外的利妥昔单抗单药治疗成功治愈。PTLD可在移植多年后发生,是一种严重的、可能危及生命的并发症。因此,早期识别和及时治疗至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4af3/11299132/42a22d43661d/cureus-0016-00000063951-i01.jpg

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