*Department of Laboratory Medicine and Pathology, Division of Hematopathology †Department of Laboratory Medicine and Pathology, Division of Molecular Diagnostics ‡Department of Laboratory Medicine and Pathology, Division of Clinical Virology, University of Minneapolis, Minneapolis, MN.
Am J Surg Pathol. 2014 Nov;38(11):1522-9. doi: 10.1097/PAS.0000000000000282.
Epstein-Barr virus (EBV)-positive mucocutaneous ulcer (EBV MCU) is a B-cell lymphoproliferative disorder occurring in elderly or iatrogenic immunocompromised patients. It has not been reported in solid organ transplant recipients. We observed 7 patients with EBV MCU in a cohort of 70 transplant recipients with EBV posttransplant lymphoproliferative disorder (PTLD). Transplants included: 5 renal, 1 heart, and 1 lung. Median patient age was 61; 5 were male. EBV MCU was observed in oral mucosa in 4 and gastrointestinal tract in 3. Duration of immunosuppressive therapy before EBV MCU was 0.6 to 13 years. Ulcers were undermined by inflammatory cells and polymorphic or monomorphic large cell lymphoproliferation. Reed-Sternberg-like cells were present in 5/7. Large B cells were CD20, CD30, and EBV-encoded RNA positive in all cases. Diagnosis in 3 recent patients was EBV MCU; 4 patients diagnosed before familiarity with EBV MCU were classified as monomorphic large cell (n=3) and polymorphic (n=1) PTLD. None of the patients had EBV DNA in their blood (<1000 copies/mL) at diagnosis or follow-up versus 35/44 transplant patients with systemic PTLD (P<0.001). All lesions resolved with reduced immunosuppression (7/7), change in immunosuppression (2/7), and rituximab (3/7). Five patients are living: 4 healthy, 1 awaiting second renal transplant. Two patients died 3 and 5 years after resolution of EBV MCU. No patient recurred with EBV MCU or other PTLDs. EBV MCU mimics more aggressive categories of PTLD but lacks EBV DNA in blood, which may be a useful distinguishing feature. Lesions are likely to resolve with conservative management. Awareness of EBV MCU in the posttransplant setting is necessary for appropriate diagnosis and treatment.
EB 病毒阳性黏膜溃疡（EBVMCU）是一种发生于老年或医源性免疫功能低下患者的 B 细胞淋巴增生性疾病。它尚未在实体器官移植受者中报道。我们在一组 70 例 EBV 移植后淋巴增生性疾病（PTLD）的移植受者中观察到 7 例 EBVMCU。移植包括：5 例肾移植、1 例心脏移植和 1 例肺移植。中位患者年龄为 61 岁；5 例男性。4 例 EBVMCU 发生于口腔黏膜，3 例发生于胃肠道。发生 EBVMCU 之前接受免疫抑制治疗的时间为 0.6 至 13 年。溃疡由炎症细胞和多形性或单形性大细胞淋巴增生浸润。7/7 例中有 Reed-Sternberg 样细胞。所有病例的大 B 细胞均为 CD20、CD30 和 EBV 编码 RNA 阳性。最近 3 例患者的诊断为 EBVMCU；4 例在认识 EBVMCU 之前诊断为单形性大细胞（n=3）和多形性（n=1）PTLD。与 44 例有全身 PTLD 的移植患者相比（P<0.001），所有患者在诊断时或随访时血液中的 EBVDNA 均<1000 拷贝/ml（n=7/7）。所有病变均通过减少免疫抑制（7/7）、改变免疫抑制（2/7）和利妥昔单抗（3/7）而缓解。5 例患者存活：4 例健康，1 例等待第二次肾移植。2 例患者在 EBVMCU 缓解后 3 年和 5 年死亡。无患者出现 EBVMCU 或其他 PTLD 复发。EBVMCU 类似于侵袭性更高的 PTLD 类别，但缺乏血液中的 EBVDNA，这可能是一个有用的鉴别特征。病变可能通过保守治疗缓解。在移植后环境中意识到 EBVMCU 的存在对于进行适当的诊断和治疗是必要的。
