以肠道病变为首发表现的成人系统性Epstein-Barr病毒阳性T细胞淋巴组织增殖性疾病

Intestinal Lesions as the Primary Clinical Manifestation of Adult-onset Systemic Epstein-Barr Virus Positive T-cell Lymphoproliferative Disease

目的提高对成人系统性Epstein-Barr(EB)病毒阳性T细胞淋巴组织增殖性疾病(ASEBV+T-LPD)肠道病变的认识。方法报道2012年3月-10月我院收治的2例以肠道病变为首发表现的ASEBV+T-LPD,并结合3例文献报道进行分析讨论。结果 2例成人患者以腹泻为主要表现,伴有发热、淋巴结长大,初期分别误诊为结核及肠道感染,淋巴结病检提示多克隆EBER+的T淋巴细胞浸润,确诊ASEBV+T-LPD。例1经3周期GLIDE方案(吉西他滨+门冬酰胺酶+异环磷酰胺+地塞米松+依托泊苷)化学疗法(化疗)后疾病进展,死于肺部感染。例2经干扰素联合抗病毒药物治疗,病情稳定。复习文献目前仅3例以肠道病变为首发表现的ASEBV+T-LPD被报道,早期均被误诊为炎性肠病或感染,3例均出现肠道穿孔或大出血等并发症,其中2例死亡。结论成人系统性EB病毒阳性T细胞淋巴组织增殖性疾病的肠道病变较为罕见,容易误诊为结核及炎性肠病。部分患者病情进展迅速,可出现肠道穿孔及消化道大出血等致死性并发症,死亡率高,预后差。确诊本病需密切结合临床和病理学检查。常规化疗及抗病毒治疗仅部分有效,不能维持长期缓解。

Objective To promote our recognition of intestinal lesions of the adult-onset systemic Epstein-Barr virus positive T-cell lymphoproliferative disease（ASEBV＋T-LPD）.Methods We reported two cases of ASEBV＋T-LPD with intestinal lesions as its primary feature diagnosed between March and October 2012 and collected the data of three cases previously reported.Results The two patients presented with chronic diarrhea and ensuing fever and lymphadenopathy,which was initially misdiagnosed as tuberculosis and other infections.Diagnosis was made by lymph node biopsy which showed polymorphic and polyclonal proliferation of EBV infected T cells.Case one died of infection and hypokalemia after three-cycle transiently effective chemotherapy of GLIDE protocol.Case two remained stable with interferon and antiviral therapy.Only three previous cases of ASEBV＋T-LPD with intestinal lesions as its primary manifestation were found by literature review.All were misdiagnosed as inflammatory bowel diseases or tuberculosis.All cases had severe complications like perforation and severe intestinal bleeding.Two of the three cases died of complications.Conclusions Intestinal lesions of ASEBV＋T-LPD are rare and can be easily misdiagnosed as tuberculosis and inflammatory bowel diseases.It can rapidly develop lethal complications like bowel perforation and severe gastrointestinal bleeding.Diagnosis of this disease should closely combine clinical manifestation with pathological study.Ordinary chemotherapy and antiviral treatment is partially effective and is unable to maintain long-term remission.