Epstein-Barr virus (EBV)-associated smooth-muscle tumor arising in a post-transplant patient treated successfully for two PT-EBV-associated large-cell lymphomas - Case report
Epstein-Barr virus (EBV)-associated smooth-muscle tumor arising in a post-transplant patient treated successfully for two PT-EBV-associated large-cell lymphomas - Case report
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DOI：
10.1097/00000478-199612000-00011
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发表时间：
1996-12-01
影响因子：
5.6
通讯作者：
Jaffe, ES
中科院分区：
文献类型：
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作者：
Kingma, DW;Shad, A;Jaffe, ES
eb病毒（EBV）与平滑肌肿瘤的关联最近在获得性免疫缺陷综合征（艾滋病）和移植后被报道。我们报告一例ebv相关的平滑肌肿瘤发生在移植后（PT）患者，他之前成功治疗了两例ebv相关的PT大细胞淋巴瘤。一名4岁女孩在23个月大时因扩张性心肌病需要心脏移植。她的治疗方案包括环孢素、氮噻唑普利和地尔硫卓。在16个月的PT时，她出现贫血，愈创木阳性大便，腹部肿块诊断为弥漫性大细胞淋巴瘤b细胞表型。减少免疫抑制治疗，开始使用干扰素和静脉注射免疫球蛋白。她迅速出现排斥反应的迹象，并进行了心脏活检，显示IIIB级排斥反应。随后，免疫抑制治疗增加。术后23个月，对盆腔大肿块行活检，诊断为免疫母细胞淋巴瘤。经化疗和维甲酸治疗后，肿块大小明显减小。随后的计算机断层扫描显示多发肝结节。肝穿刺活检显示一个平滑肌肿瘤，级别不确定。经Epstein-Barr （EBER1）原位杂交检测，淋巴瘤和平滑肌瘤细胞中EBV含量均在95%以内，Epstein-Barr核抗原-2 （EBNA-2）聚合酶链反应（PCR）检测均为A型，PCR分析发现潜伏膜蛋白(LMP)-1癌基因缺失30个碱基对（346-355个氨基酸）。值得注意的是，最初的大细胞淋巴瘤和随后的免疫母细胞淋巴瘤都包含一个独特的p53突变，这表明它们是不同的。这些数据表明，同一种病毒导致了恶性淋巴瘤和平滑肌瘤的发病机制。
The association of Epstein-Barr virus (EBV) with smooth-muscle tumors was recently reported in the setting of acquired immunodeficiency syndrome (AIDS) and post-transplantation. We report a case of an EBV-associated smooth-muscle tumor arising in a post-transplant (PT) patient who previously was treated successfully for two EBV-associated PT large-cell lymphomas. A 4-year-old girl required cardiac transplantation for dilated cardiomyopathy when she was aged 23 months. Her PT regimen included cyclosporine, azothiaprine, and diltiazem. At 16 months PT, she presented with anemia, guaiac-positive stools, and an abdominal mass diagnosed as diffuse large-cell lymphoma of B-cell phenotype. Immunosuppressive therapy was reduced, and interferon and i.v. immunoglobulin were initiated. She rapidly developed signs of rejection, and a cardiac biopsy was performed, revealing grade IIIB rejection. Subsequently, immunosuppressive therapy increased. At 23 months PT, a biopsy was done of a large pelvic mass that was diagnosed as immunoblastic large-cell lymphoma After treatment with chemotherapy and retinoic acid, the size of the mass markedly decreased. Follow-up computed tomography scan revealed multiple liver nodules. A needle biopsy of the liver showed a smooth-muscle tumor of indeterminate grade. Both the lymphomas and the smooth-muscle tumor contained EBV within >95% of tumor cells by Epstein-Barr (EBER1) in situ hybridization, were of strain type A by Epstein-Barr nuclear antigen-2 (EBNA-2) polymerase chain reaction (PCR) and contained an identical 30 base-pair deletion (amino acids 346-355) of the latent membrane protein (LMP)-1 oncogene by PCR analysis. Notably, the initial large-cell lymphoma and the subsequent immunoblastic lymphoma each contained a unique p53 mutation, suggesting that they were distinct. These data suggest that the same virus contributed to the pathogenesis of both the malignant lymphomas and the smooth-muscle tumor.