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.
EBV阳性皮肤黏膜溃疡(EBV MCU)是一种B细胞增殖性疾病，发生于老年或医源性免疫抑制患者。在实体脏器移植患者中还没有被报道。我们在一组70例患有EBV移植后淋巴组织增生性疾病 (PTLD)患者中观察到7例EBV MCU患者。移植者包括：5例肾，1例心脏，1例肺。中位年龄61岁， 5例男性。EBV MCU4例出现于口腔黏膜，胃肠道3例。EBV MCU以前接受免疫抑制治疗时间为0.6到 13年。溃疡由炎细胞和多形性或单形性大细胞增生构成。5/7例出现Reed-Sternberg样细胞。所有病例中大B 细胞表达CD20, CD30,和EBV-encoded RNA。3例近期病例诊断为EBV MCU; 4例曾被诊断为单形性大细胞PTLD (n=3)和多形性 PTLD (n=1)。.诊断时或随访血中均未检出EBV DNA (<1000 copies/mL)，而35/44系统性PTLD移植患者则检出 (P<0.001)。患者全部治愈，分别为降低免疫抑制(7/7)，改变免疫抑制剂 (2/7)和美罗华治疗 (3/7)。 5例患者存活：4例健康，1例等待第二次肾移植。2例患者分别于EBV MCU治愈后3年和5年死亡。没有患者复发EBV MCU或其它PTLDs. EBV MCU可类似更具侵袭性的PTLD，但前者血中缺少EBV DNA，是一个很有用的鉴别诊断特点。通过保守治疗可以达到病变的治愈。在移植后患者认识EBV MCU对于恰当的诊断和治疗该疾病是必要的。