Epstein-Barr virus-driven hemophagocytic lymphohistiocytosis

doi:10.3969/j.issn.1004-583X.2021.10.009

Abstract

Abstract:

Hemophagocytic lymphohistiocytosis (HLH) is the life-threatening result of inflammatory storm caused by immunological dysfunction. With the development of molecular genetics, current studies have an in-depth understanding of the HLH. For some genotype of HLH, epstein-barr virus (EBV) is an important inducement, known as the EBV-driven HLH. Currently, the Tyrosine-protein kinase ITK deficiency, magnesium transporter protein 1 (MAGT1) deficiency, CD27 deficiency, CD70 deficiency, CTP synthase 1 (CTPS1) deficiency and RAS guanyl-releasing protein 1 (RASGRP1) deficiency are found to be the genotype related to the HLH. In this review, we would summarize the epidemiology, pathophysiological characteristics, clinical manifestations and treatment of EBV-driven HLH.

Key words: lymphohistiocytosis,hemophagocytic, herpesvirus 4,human

CLC Number:

R551.12

Cui Tingting, Zhang Jia, Wang Jingshi, Wang Zhao. Epstein-Barr virus-driven hemophagocytic lymphohistiocytosis[J]. Clinical Focus, 2021, 36(10): 910-915.

Add to citation manager EndNote|Ris|BibTeX

URL: https://huicui.hebmu.edu.cn/EN/10.3969/j.issn.1004-583X.2021.10.009

https://huicui.hebmu.edu.cn/EN/Y2021/V36/I10/910

References 23

[1]	Canna SW, Marsh RA. Pediatric hemophagocytic lymphohistiocytosis[J]. Blood, 2020,135(16):1332-1343. doi: 10.1182/blood.2019000936 URL
[2]	韩月恒, 药立波, 苏成芝. Tec家族蛋白酪氨酸激酶Itk的研究进展[J]. 上海免疫学杂志, 2000,20(2):125-126.
[3]	Lechner KS, Neurath MF, Weigmann B. Role of the IL-2 inducible tyrosine kinase ITK and its inhibitors in disease pathogenesis[J]. J Mol Med (Berl), 2020,98(10):1385-1395. doi: 10.1007/s00109-020-01958-z URL
[4]	Ghosh S, Drexler I, Bhatia S, et al. Interleukin-2-inducible T-cell kinase deficiency-new patients, new insight?[J]. Front Immunol, 2018,9:979. doi: 10.3389/fimmu.2018.00979 URL
[5]	Kapnick SM, Stinchcombe JC, Griffiths GM, et al. Inducible T cell kinase regulates the acquisition of cytolytic capacity and degranulation in CD8+ CTLs[J]. J Immunol, 2017,198(7):2699-2711. doi: 10.4049/jimmunol.1601202 pmid: 28213500
[6]	Linka RM, Risse SL, Bienemann K, et al. Loss-of-function mutations within the IL-2 inducible kinase ITK in patients with EBV-associated lymphoproliferative diseases[J]. Leukemia, 2012,26(5):963-971. doi: 10.1038/leu.2011.371 pmid: 22289921
[7]	Ghosh S, Bienemann K, Boztug K, et al. Interleukin-2-inducible T-cell kinase (ITK) deficiency - clinical and molecular aspects[J]. J Clin Immunol, 2014,34(8):892-899. doi: 10.1007/s10875-014-0110-8 URL
[8]	Ravell JC, Chauvin SD, He T, Lenardo M. An Update on XMEN Disease[J]. J Clin Immunol, 2020,40(5):671-681. doi: 10.1007/s10875-020-00790-x pmid: 32451662
[9]	Ravell JC, Matsuda-Lennikov M, Chauvin SD, et al. Defective glycosylation and multisystem abnormalities characterize the primary immunodeficiency XMEN disease[J]. J Clin Invest, 2020,130(1):507-522. doi: 10.1172/JCI131116 pmid: 31714901
[10]	Ravell J, Chaigne-Delalande B, Lenardo M. X-linked immunodeficiency with magnesium defect, Epstein-Barr virus infection, and neoplasia disease: a combined immune deficiency with magnesium defect[J]. Curr Opin Pediatr, 2014,26(6):713-719. doi: 10.1097/MOP.0000000000000156 pmid: 25313976
[11]	Li FY, Chaigne-Delalande B, Su H, et al. XMEN disease: a new primary immunodeficiency affecting Mg2+ regulation of immunity against Epstein-Barr virus[J]. Blood, 2014,123(14):2148-2152. doi: 10.1182/blood-2013-11-538686 URL
[12]	王雷雷, 孔英君. CD70-CD27通路在肿瘤发生及治疗中作用的研究进展[J]. 临床与病理杂志, 2019,39(9):2021-2025.
[13]	Veillette A, Perez-Quintero LA, Latour S. X-linked lymphoproliferative syndromes and related autosomal recessive disorders[J]. Curr Opin Allergy Clin Immunol, 2013,13(6):
[14]	Latour S, Winter S. Inherited immunodeficiencies with high predisposition to epstein-barr virus-driven lymphoproliferative diseases[J]. Front Immunol, 2018,9:1103. doi: 10.3389/fimmu.2018.01103 URL
[15]	Tangye SG, Palendira U, Edwards ES. Human immunity against EBV-lessons from the clinic[J]. J Exp Med, 2017,214(2):269-283. doi: 10.1084/jem.20161846 URL
[16]	Izawa K, Martin E, Soudais C, et al. Inherited CD70 deficiency in humans reveals a critical role for the CD70-CD27 pathway in immunity to Epstein-Barr virus infection[J]. J Exp Med, 2017,214(1):73-89. doi: 10.1084/jem.20160784 URL
[17]	Ghosh S, Bal SK, Edwards ESJ, et al. Extended clinical and immunological phenotype and transplant outcome in CD27 and CD70 deficiency[J]. Blood, 2020,136(23):2638-2655. doi: 10.1182/blood.2020006738 URL
[18]	Alkhairy OK, Perez-Becker R, Driessen GJ, et al. Novel mutations in TNFRSF7/CD27: Clinical, immunologic, and genetic characterization of human CD27 deficiency[J]. J Allergy Clin Immunol, 2015,136(3):703-712 e710. doi: 10.1016/j.jaci.2015.02.022 pmid: 25843314
[19]	Wang H, Kaur G, Sankin AI, et al. Immune checkpoint blockade and CAR-T cell therapy in hematologic malignancies[J]. J Hematol Oncol, 2019,12(1):59. doi: 10.1186/s13045-019-0746-1 URL
[20]	Martin E, Palmic N, Sanquer S, et al. Erratum: CTP synthase 1 deficiency in humans reveals its central role in lymphocyte proliferation[J]. Nature, 2014,511(7509):370-370.
[21]	Cohen JI. Primary immunodeficiencies associated with ebv disease[J]. Curr Top Microbiol Immunol, 2015,390(Pt 1):241-265.
[22]	Kucuk ZY, Zhang K, Filipovich L, et al. CTP Synthase 1 deficiency in successfully transplanted siblings with combined immune deficiency and chronic active EBV infection[J]. J Clin Immunol, 2016,36(8):1-4.
[23]	Somekh I, Marquardt B, Liu Y, et al. Novel mutations in RASGRP1 are associated with immunodeficiency, immune dysregulation, and EBV-induced lymphoma[J]. J Clin Immunol, 2018,38(6):699-710. doi: 10.1007/s10875-018-0533-8 pmid: 30030704