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一名肾移植患者中与爱泼斯坦-巴尔病毒相关的移植后平滑肌肿瘤

Epstein-Barr virus-associated post-transplant smooth muscle tumours in a kidney transplant patient.

作者信息

Cobeñas Carlos J, Pereyra Priscila, Spizzirri Ana Paula, Gauto Santacruz Cristina, Del Carmen Suarez Angela, Altamirano Eugenia, Pérez Paula, Tello Nicolas

机构信息

Nephrology Department, Hospital de Niños "Sup. Sor María Ludovica", La Plata, Buenos Aires, Argentina.

Pathology Department, Hospital de Niños "Sup. Sor María Ludovica", La Plata, Argentina.

出版信息

Pediatr Nephrol. 2025 Feb;40(2):389-391. doi: 10.1007/s00467-024-06493-4. Epub 2024 Sep 4.

DOI:10.1007/s00467-024-06493-4
PMID:39230732
Abstract

We report on a 14-year-old girl who developed post-transplantation smooth muscle tumours (PTSMT) located in the spleen, lungs, liver, and central nervous system (CNS), 4 years after kidney transplantation. She was asymptomatic, and the disease was detected during the work-up for a urinary tract infection. Diagnosis was performed by the analysis of a tissue specimen, through the biopsy of a lung tumour, which revealed a proliferation of spindle-shaped cells which were positive for actin and vimentin. In situ hybridization studies were positive for Epstein-Barr virus, and her serologic status was negative prior to transplantation. We reduced immunosuppression by stopping mycophenolate and switching tacrolimus for sirolimus. After 18 months of follow-up, she remains asymptomatic, and the CNS tumour reduced its diameter from 24 × 21 mm to 14 × 13 mm. PTSMT should be considered in the differential diagnosis of transplanted patients who develop neoplastic complications associated with immunosuppression.

摘要

我们报告了一名14岁女孩,她在肾移植4年后出现了位于脾脏、肺、肝脏和中枢神经系统(CNS)的移植后平滑肌肿瘤(PTSMT)。她没有症状,该病是在对尿路感染进行检查时被发现的。通过对肺肿瘤活检的组织标本进行分析来进行诊断,结果显示梭形细胞增殖,肌动蛋白和波形蛋白呈阳性。原位杂交研究显示爱泼斯坦-巴尔病毒呈阳性,且她在移植前的血清学状态为阴性。我们通过停用霉酚酸酯并将他克莫司换为西罗莫司来减少免疫抑制。经过18个月的随访,她仍然没有症状,中枢神经系统肿瘤的直径从24×21毫米缩小到了14×13毫米。对于出现与免疫抑制相关的肿瘤并发症的移植患者,鉴别诊断时应考虑PTSMT。

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本文引用的文献

1
Epstein-Barr virus-associated smooth muscle tumours after transplantation, infection with human immunodeficiency virus and congenital immunodeficiency syndromes.移植后与 Epstein-Barr 病毒相关的平滑肌肿瘤、人类免疫缺陷病毒感染和先天性免疫缺陷综合征。
Pathobiology. 2013;80(6):297-301. doi: 10.1159/000351326. Epub 2013 Aug 30.
2
Expression of EBV latent antigens, mammalian target of rapamycin, and tumor suppression genes in EBV-positive smooth muscle tumors: clinical and therapeutic implications.EBV 潜伏抗原、雷帕霉素哺乳动物靶点及肿瘤抑制基因在 EBV 阳性平滑肌瘤中的表达:临床及治疗意义
Clin Cancer Res. 2009 Sep 1;15(17):5350-8. doi: 10.1158/1078-0432.CCR-08-2979. Epub 2009 Aug 25.