Intestinal Lesions as the Primary Clinical Manifestation of Adult-onset Systemic Epstein-Barr Virus Positive T-cell Lymphoproliferative Disease_West China Medical Journal

Authors：

SHEN Kai , CHEN Xinchuan ,  LIU Ting.

Department of hematology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;

Corresponding author：

LIU Ting., Email: 453038222@qq.com

Keywords：

Epstein-Barr病毒感染; 淋巴细胞增殖性疾病; T淋巴细胞; 肠道淋巴瘤

DOI：

10.7507/1002-0179.20130264

Video：

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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。

Citation： SHEN Kai,CHEN Xinchuan,LIU Ting.. Intestinal Lesions as the Primary Clinical Manifestation of Adult-onset Systemic Epstein-Barr Virus Positive T-cell Lymphoproliferative Disease. West China Medical Journal, 2013, 28(6): 840-843. doi: 10.7507/1002-0179.20130264 Copy

1.	Cohen JI, Kimura H, Nakamura S, et al. Epstein-Barr virus-associated lymphoproliferative disease in non-immunocompromised hosts: a status report and summary of an international meeting, 8-9 September 2008[J]. Ann Oncol, 2009, 20(9): 1472-1482.
2.	Swerdlow SH, Campo E, Harris NL, et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues[M]. Lyon: IARC, 2008: 278-280.
3.	Kimura H, Ito Y, Kawabe S, et al. EBV-associated T/NK-cell lymphoproliferative diseases in nonimmunocompromised hosts: prospective analysis of 108 cases[J]. Blood, 2012, 119(3): 673-686.
4.	Abdul-Ghafar J, Kim JW, Park KH, et al. Fulminant Epstein-Barr virus-associated T-cell lymphoproliferative disorder in an immunocompetent middle-aged man presenting with chronic diarrhea and gastrointestinal bleeding[J]. J Korean Med Sci, 2011, 26(8): 1103-1107.
5.	Park S, Kim K, Kim WS, et al. Systemic EBV+ T-cell lymphoma in elderly patients: comparison with children and young adult patients[J]. Virchows Arch, 2008, 453(2): 155-163.
6.	Isobe Y, Aritaka N, Setoguchi Y, et al. T/NK cell type chronic active EpsteineBarr virus disease in adults: an underlying condition for EpsteineBarr virus-associated T/NK-cell lymphoma[J]. J Clin Pathol, 2012, 65(3): 278-282.
7.	郑晓丹, 周小鸽, 金妍, 等. 成年人系统性EB病毒阳性T/NK细胞淋巴组织增殖性疾病的临床病理研究[J]. 中华病理学杂志, 2011, 40(4): 227-234.
8.	Ohshima K, Kimura H, Yoshino T, et al. Proposed categorization of pathological states of EBV-associated T/natural killer-cell lymphoproliferative disorder (LPD) in children and young adults: overlap with chronic active EBV infection and infantile fulminant EBV T-LPD[J]. Pathol Int, 2008, 58(4): 209-217.
9.	Lee YJ, Yang SK, Byeon JS, et al. Analysis of colonoscopic findings in the differential diagnosis between intestinal tuberculosis and Crohn’s disease[J]. Endoscopy, 2006, 38(6): 592-597.
10.	渡部优子, 高桥真由美, 三浦修, 等.成人EB病毒阳性T/NK细胞淋巴组织增殖性疾病的临床特征及化疗效果[C].第20届EB病毒感染性疾病研究会学术会议论文汇编, 东京, 2010. 东京: EB病毒感染性疾病研究会, 2010.
11.	Arai A, Imadome K, Watanabe Y, et al. Clinical features of adult-onset chronic active Epstein-Barr virus infection: a retrospective analysis[J]. Int J Hematol, 2011, 93(5): 602-609.
12.	Kawa K, Sawada A, Sato M, et al. Excellent outcome of allogeneic hematopoietic SCT with reduced-intensity conditioning for the treatment of chronic active EBV infection[J]. Bone Marrow Transplant, 2011, 46(1): 77-83.

1. 　Cohen JI, Kimura H, Nakamura S, et al. Epstein-Barr virus-associated lymphoproliferative disease in non-immunocompromised hosts: a status report and summary of an international meeting, 8-9 September 2008[J]. Ann Oncol, 2009, 20(9): 1472-1482.
2. 　Swerdlow SH, Campo E, Harris NL, et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues[M]. Lyon: IARC, 2008: 278-280.
3. 　Kimura H, Ito Y, Kawabe S, et al. EBV-associated T/NK-cell lymphoproliferative diseases in nonimmunocompromised hosts: prospective analysis of 108 cases[J]. Blood, 2012, 119(3): 673-686.
4. 　Abdul-Ghafar J, Kim JW, Park KH, et al. Fulminant Epstein-Barr virus-associated T-cell lymphoproliferative disorder in an immunocompetent middle-aged man presenting with chronic diarrhea and gastrointestinal bleeding[J]. J Korean Med Sci, 2011, 26(8): 1103-1107.
5. 　Park S, Kim K, Kim WS, et al. Systemic EBV+ T-cell lymphoma in elderly patients: comparison with children and young adult patients[J]. Virchows Arch, 2008, 453(2): 155-163.
6. 　Isobe Y, Aritaka N, Setoguchi Y, et al. T/NK cell type chronic active EpsteineBarr virus disease in adults: an underlying condition for EpsteineBarr virus-associated T/NK-cell lymphoma[J]. J Clin Pathol, 2012, 65(3): 278-282.
7. 　郑晓丹, 周小鸽, 金妍, 等. 成年人系统性EB病毒阳性T/NK细胞淋巴组织增殖性疾病的临床病理研究[J]. 中华病理学杂志, 2011, 40(4): 227-234.
8. 　Ohshima K, Kimura H, Yoshino T, et al. Proposed categorization of pathological states of EBV-associated T/natural killer-cell lymphoproliferative disorder (LPD) in children and young adults: overlap with chronic active EBV infection and infantile fulminant EBV T-LPD[J]. Pathol Int, 2008, 58(4): 209-217.
9. 　Lee YJ, Yang SK, Byeon JS, et al. Analysis of colonoscopic findings in the differential diagnosis between intestinal tuberculosis and Crohn’s disease[J]. Endoscopy, 2006, 38(6): 592-597.
10. 　渡部优子, 高桥真由美, 三浦修, 等.成人EB病毒阳性T/NK细胞淋巴组织增殖性疾病的临床特征及化疗效果[C].第20届EB病毒感染性疾病研究会学术会议论文汇编, 东京, 2010. 东京: EB病毒感染性疾病研究会, 2010.
11. 　Arai A, Imadome K, Watanabe Y, et al. Clinical features of adult-onset chronic active Epstein-Barr virus infection: a retrospective analysis[J]. Int J Hematol, 2011, 93(5): 602-609.
12. 　Kawa K, Sawada A, Sato M, et al. Excellent outcome of allogeneic hematopoietic SCT with reduced-intensity conditioning for the treatment of chronic active EBV infection[J]. Bone Marrow Transplant, 2011, 46(1): 77-83.

West China Medical Journal

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

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