淋巴瘤相关噬血细胞综合征临床特点

Abstract
Figure/Table
References (18)
Related Citation (4)

Download: PDF (435 KB) HTML (1 KB)
Export: BibTeX | EndNote (RIS)

Abstract Objective To investigate the clinical characteristics of lymphoma associated hemophagocytic syndrome. Methods The clinical data of 18 patients with lymphoma associated hemophagocytic syndrome admitted to Shantou Central Hospital from January 2012 to June 2017 was analyzed retrospectively. Results Among 18 patients, there were 13 cases of patients with T cell lymphoma, and the main clinical stages were moderate and advanced stages. There was no significant difference of pathological types of lymphoma in different gender (χ2 = 0.628, P ＞ 0.05). The clinical stage of lymphoma was positively correlated with the age of onset (r = 0.765, P ＜ 0.05). The older the patients were, the later the clinical stage of lymphoma was. The related gene detection of lymphoma associated hemophagocytic syndrome showed that there were 9 cases of gene mutation, among which, 6 cases had UNC13D mutations. According to hemophagocytic syndrome-94 regimen, all patients were given glucocorticoid and Etoposide to improve hemophagocytic symptoms and then gradually transferred to chemotherapy. 13 of 18 patients died. EB virus infection and severe thrombocytopenia were the major causes of death of lymphoma related hemophagocytic syndrome. Conclusion Lymphoma related hemophagocytic syndrome has a wide variety of clinical manifestations and high mortality. Most patients have EB virus infection. Early diagnosis and treatment is the key.

Key words： Lymphoma associated hemophagocytic syndrome Clinical features EB virus Gene mutation

	Service

	E-mail this article
	Add to my bookshelf
	Add to citation manager
	E-mail Alert
	RSS
	Articles by authors
	LI Yongqian1 CHEN Sushan1 CHEN Manna1 CAI Songhao1 YE Junbin1 YANG Qimei1 DU Zepeng2 HONG Shaojie1

Cite this article:

LI Yongqian1 CHEN Sushan1 CHEN Manna1 CAI Songhao1 YE Junbin1 YANG Qimei1 DU Zepeng2 HONG Shaojie1. Clinical characteristics of lymphoma associated hemophagocytic syndrome[J]. 中国医药导报, 2019, 16(5): 63-67.

URL:

http://www.yiyaodaobao.com.cn/EN/ OR http://www.yiyaodaobao.com.cn/EN/Y2019/V16/I5/63

[1] 王昭.如何诊断噬血细胞综合征[J].中华血液学杂志，2016，37（7）：550-553.
[2] Jaffe ES. The 2008 WHO classification of lymphomas：implications for clinical practice and translational research [J]. Hemotol Am Soc Hematol Educ Program，2009：523-531.
[3] Henter JI，Horne A，Aricó M，et al. HLH-2004：Diagnostic and therapeutic guideline for hemophagocytic lymphohistiocytosis [J]. Pediatr Blood Cancer，2007，48（2）：124-131.
[4] Henter JI，Aricò M，Egeler RM，et al. HLH-94：a treatment protocol for hemophagocytic lymphohistiocytosis. HLH study Group of the Histiocyte Society [J]. Med Pediater Oncol，1997，28（5）：342-347.
[5] Usmani GN，Woda BA，Newburger PE. Advances in understanding the pathogenesis of HLH [J]. Br J Haematol，2013，161（5）：609-622.
[6] Wang Y，Wang Z，Zhang J，et al. Genetic features of late onset primary hemophagocytic lymphohistiocytosis in adolescence or adulthood [J]. PLoS One，2014，9（9）：e107386.
[7] 李硕，王晶石，王旖旎，等.147例噬血细胞综合征病因及预后分析[J].临床血液学杂志，2014，27（7）：559-563.
[8] 中国抗癌协会淋巴瘤专业委员会.淋巴瘤相关噬血细胞综合征诊治中国专家共识[J].中华医学杂志，2018，98（18）：1389-1393.
[9] Hayden A，Park S，Giustini D，et al. Hemophagocytic syndromes （HPSs）including hemophagocytic lymphohistiocytosis（HLH）in adults：A systematic scoping review [J]. Blood Rev，2016，30（6）：411-420.
[10] Revière S，Galicier L，Coppo P，et al. Reactive hemophagocytic syndrome in adults：a retrospective analysis of 162 patients [J]. Am J Med，2014，127（11）：1118-1125.
[11] Ramos-Casals M，Brito-Zerón P，López-Guillermo A，et al. Adult haemophagocytic syndrome [J]. Lancet，2014， 383（9927）：1503-1516.
[12] Zhang K，Chandrakasan S，Chapman H，et al. Synergistic defects of different molecules in the cytotoxic pathway lead to clinical familial hemophagocytic lymphohistiocytosis [J]. Blood，2014，124（8）：1331-1334.
[13] 牛钰，何爱丽，张玉刚.嗜血细胞性淋巴组织细胞增生症研究进展[J].中国实验血液学杂志，2010，18（1）：262-267.
[14] Clementi R，Locatelli F，Dupre L，et al. A proportion of patients with lymphoma may harbor mutations of the perforin gene [J]. Blood，2005，105（11）：4424-4428.
[15] Katano H，Ali MA，Patera AC，et al. Chronic active Epstein-Barr virus infection associated with mutations in perforin that impair its maturation [J]. Blood，2004，103（5）：1244-1252.
[16] Fukaya S，Yasuda S，Hashimoto T，et al. Clinical features of haemophagocytic syndrome in patients with systemic autoimmune disease：analysis of 30 cases [J]. Rheumatology（Oxford），2008，47（11）：1686-1691.
[17] Allen CE，McClain KL. Pathophysiology and epidemiology of hemophagocytic lymphohistiocytosis [J]. Hematology Am Soc Hematol Educ Program，2015，2015：177-182.
[18] Wang Y，Huang W，Hu L，et al. Multicenter study of combination DEP regimen as a salvage therapy for adult refractory hemophagocytic lymphohistiocytosis [J]. Blood，2015，126（19）：2186-2192.