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Clinical and pathological analysis of systemic Epstein-Barr virus positive T-cell lymphoma of childhood:A case report and literature review
Received date: 2018-10-17
Online published: 2020-02-25
Objective: To investigate the clinicopathological characteristics of systemic Epstein-Barr virus (EBV) positive T-cell lymphoma of childhood and improve the understanding of the new diagnostic criteria. Methods: Clinical presentations, pathological characteristics, gene detection results and immunohistochemical phenotyping of biopsy tissue from a case of systemic EBV positive T-cell lymphoma of childhood were analyzed and the relevant literature was reviewed. Result: The 11-year-old boy with prolonged mite allergy treatment and no responsive rash on limbs and torso presented recurrent high fever, generalized lymphadenopathy and hemophagocytic syndrome. Lymph node biopsy revealed diffuse proliferation of small to intermediate neoplastic T cells, which was possibly originated in interfollicular area and positive for CD3(+++), CD4(+), TIA-1(+++), GranzymeB(+++) and MIB-1 (>80% positive). The in situ hybridization of EBV (EBER) was positive (+++) and clonal TCRγ gene rearrangemen was also detected. The diagnosis of systemic EBV positive T-cell lymphoma was thus established. Conclusions: Systemic EBV-positive T-cell lymphoma of childhood is a rare systemic illness characterized by clonal proliferation of EBV-infected T cells and aggressive and fulminant clinical course often associated with hemophagocytic syndrome, which requires differentiation with medical conditions such as invasive NK cells, leukemia/lymphoma, EBV infection associated lymphoproliferative lesions and primary cutaneous gamma-delta T-cell lymphoma.
LI Qinqin, JIN Xiaolong, YUAN Fei . Clinical and pathological analysis of systemic Epstein-Barr virus positive T-cell lymphoma of childhood:A case report and literature review[J]. Journal of Diagnostics Concepts & Practice, 2020 , 19(1) : 63 -68 . DOI: 10.16150/j.1671-2870.2020.01.013
[1] | Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms[J]. Blood,2016, 127(20):2375-2390. |
[2] | Hong M, Ko YH, Yoo KH, et al. EBV-positive T/NK-cell lymphoproliferative disease of childhood[J]. Korean J Pathol, 2013, 47(2):137-147. |
[3] | 周小鸽, 张燕林, 谢建兰, 等. 对 EB 病毒相关淋巴组织增殖性疾病的理解和认识对[J]. 中华病理学杂志, 2016, 45(12):817-821. |
[4] | Virelizier JL, Lenoir G, Griscelli C. Persistent Epstein-Barr virus infection in a child with hypergammaglobulinaemia and immunoblastic proliferation associated with a selective defect in immune interferon secretion[J]. Lancet, 1978, 2(8083):231-234. |
[5] | Quintanilla-Martinez L, Kimura H, Jaffe ES, et al. EBV-positive T-cell lymphoproliferative disorders of childhood fM1[M]// Swerdlow S, Campo E, Harris NL, et al. World Health Organization classification of tumors. WHO classification of tumours of haematopoi eric and lymphoid tissues. France: IARC Press, 2008:278-280. |
[6] | Sevilla DW, El-Mallawany NK, Emmons FN, et al. Spectrum of childhood Epstein-Barr virus-associated T-cell proliferations and bone marrow findings[J]. Pediatr Dev Pathol, 2011, 14(1):28-37. |
[7] | Rodríguez-Pinilla SM, Barrionuevo C, García J, et al. Epstein-Barr virus-positive systemic NK/T-cell lymphomas in children: report of six cases[J]. Histopathology, 2011, 59(6):1183-1193. |
[8] | Xiao HJ, Li J, Song HM, et al. Epstein-Barr virus-positive T/NK-cell lymphoproliferative disorders manifested as gastrointestinal perforations and skin lesions: A case report[J]. Medicine (Baltimore), 2016, 95(5):e2676. |
[9] | Kimura H, Hoshino Y, Kanegane H, et al. Clinical and virologic characteristics of chronic active Epstein-Barr virus infection[J]. Blood. 2001 Jul 15; 98(2):280-286. |
[10] | Kimura H, Morishima T, Kanegane H, et al. Prognostic factors for chronic active Epstein-Barr virus infection[J]. J Infect Dis, 2003, 187(4):527-533. |
[11] | 金妍, 周小鸽, 何乐健, 等. 儿童系统性EB病毒阳性T细胞淋巴组织增殖性疾病的临床病理观察[J]. 中华病理学杂志, 2009, 38(9):600-608. |
[12] | Okano M, Kawa K, Kimura H, et al. Proposed guidelines for diagnosing chronic active Epstein-Barr virus infection[J]. Am J Hematol, 2005, 80(1):64-69. |
[13] | 王杰松, 杨瑜, 何鸿鸣, 等. P-Gemox方案与P-GDP方案治疗NK/T细胞淋巴瘤的疗效比较[J]. 安徽医学, 2018, 39(10):1202-1206 |
[14] | 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. |
[15] | 王微, 遆红娟, 农琳, 等. 儿童系统性EB病毒阳性T细胞淋巴增殖性疾病临床病理学分析[J]. 北京大学学报:医学版, 2012, 44(4):594-598. |
[16] | Leeborg N, Russell T, Fan G. Systemic Epstein-Barr virus positive T-cell lymphoproliferative disease of childhood[J]. Pathol Case Rev, 2012, 17:120-124. |
[17] | Kimura H, Hoshino Y, Kanegane H, et al. Clinical and virologic characteristics of chronic active Epstein-Barr virus infection[J]. Blood, 2001, 98(2):280-286. |
[18] | Sato E, Ohga S, Kuroda H, et al. Allogeneic hematopoie-tic stem cell transplantation for Epstein-Barr virus-associated T/natural killer-cell lymphoproliferative disease in Japan[J]. Am J Hematol, 2008, 83(9):721-727. |
[19] | 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. |
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