噬血细胞综合征的临床研究进展_West China Medical Journal

Authors：

刘潇潇 ,  练颖

Corresponding author：

练颖, Email: pure_m@163.com

Keywords：

噬血细胞综合征; 穿孔素; 细胞因子; HLH-2004治疗方案

DOI：

CNKI: 51-1356/R.20120214.1340.011

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噬血细胞综合征是由淋巴细胞、单核细胞和吞噬细胞系统异常激活、增殖所引起，由此导致的高细胞因子血症及继起的过度炎症反应，以致骨髓或者淋巴组织、器官中出现异常增多的组织细胞，并伴有吞噬自身血细胞行为为特征的综合征。其病因多样、临床表现复杂，如不及时治疗，患者可很快死于感染及多器官衰竭。现将近年来对噬血细胞综合征的临床研究进行综述。

Citation： 刘潇潇,练颖. 噬血细胞综合征的临床研究进展. West China Medical Journal, 2012, 27(2): 203-205. doi: CNKI: 51-1356/R.20120214.1340.011 Copy

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18.	方涵, 谢军军, 王剑超. 噬血细胞综合征患者血清LDH和β 2 -MG检测的临床意义[J]. 检验医学, 2010, 25(10): 809-807.
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22.	Filipovich AH. Life-threatening hemophagocytic syndromes: current outcomes with hematopoietic stem cell transplantation[J]. Pediatr transplant, 2005, 9(Suppl 7): 87-91.

1. Verbsky JW, Grossman WJ. Hemophagoeytic lymphohistiocytosis: diagnosis, pathophysiology, treatment and future perspectives[J]．Ann Med, 2006, 38(1): 20-31．.
2. Mien M, De Fusco C, Legrand F, et a1. Familial hemophagocytic lymphohistiecytosi: how late can the onset be?[J]. Haenlatologica, 2001, 86(5): 499-503.
3. Arie M, Danesino C, Pende D, et a1. Pathogenesis of haemophagoeytic lymphohistiocytosis[J]. Br J Haematol, 2001, l14(3): 761-769.
4. Rudd E, Göransdotter Ericson K, Zheng C, et a1. Spectrum and clinical implications of syntaxin ll gene mutations in familial haemophagocytic lymphohistiocytosis：association with disease-free remissions and haematopoietic malignancies[J]. J Med Genet, 2006, 43(4): 14.
5. Schneider EM, Lorenz L, Muller-Rosenberger M, et a1. Hemophagoeytic v lymphohistiecytosis is associated with deficiencies of cdluhr cytolysis but norlnal expression of transcripts relevant to killer-cell-induced apoptesis[J]. Blood, 2002, 100(11): 2891-2898.
6. de Saint Basile G, Fischer A. Defective cytotoxic granule-mediatedcell death pathway impairs T lymphocyte homeostasis[J]. Curr Opin Rheumatoid, 2003, 15(4): 436-445.
7. Janka G. Familial and acquired hemophagocytie lymphohistioeytosis[J]. Eur J Pediatr, 2007, 166(2): 95-109.
8. Henter JI, HonIe AC, Egeler RM, et a1. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis[J]. Pediatr B1ood Cancer, 2007(48): 124-131.
9. 张炯, 邹和建. 自身免疫性疾病并发反应性噬血细胞综合征2例[J]. 内科理论与实践, 2008, 3(3): 190-192.
10. 皇甫春荣, 付红敏. EB病毒相关性噬血细胞综合征的临床特征及外周血淋巴细胞亚群变化[J]. 中国小儿急救医学, 2010, 17(4): 330-332.
11. Janka G. Hemophagucytie syndromes[J]. Blood Rev, 2007, 21(5): 245-253.
12. 王学文. 噬血细胞综合征的研究进展[J]. 临床肿瘤学杂志, 2008, 13(11): 1042-1046.
13. Larroche C, Mouthon L. Pathogenesis of hemophagocytic syndrome (HPS)[J]. Autoimmun Rev, 2004, 3(2): 69-75.
14. Quintanilla ML, Kumar S, Fend F, et a1. Fulminant EBV(+)T-cell lymphoproliferative disorder following acute/chronic EBV infection：a distinct clinicopathologic syndrome[J]. Blood, 2000, 96(2): 443-451.
15. Filipevich AH. Hemophagocytie lymphohistiocytosis (HLH) and related disorders[M]. Hematology Am Soc Hematol Educ Program, 2009, 1(1): 127-131.
16. 黄道连, 鞠文东, 付四毛, 等. 噬血细胞综合征早期实验室指标改变研究[J]. 实验与检验医学, 2008, 26(6): 598-599.
17. 李芳秋. 噬血细胞综合征发病机制及实验诊断[J]. 临床检验杂志, 2004, 22(1): 532-533.
18. 方涵, 谢军军, 王剑超. 噬血细胞综合征患者血清LDH和β 2 -MG检测的临床意义[J]. 检验医学, 2010, 25(10): 809-807.
19. Zidan J, Hussein O, Basher W , et al. Serum CA125: a tumor marker for monitoring response to treatment and follow-up in patients with non-Hodgkin’s lymphoma[J]. Oncologist, 2004, 9(4): 417-421.
20. Wang Z, Wang Y, Feng C, et al. Early diagnosis and treatment 0f the patients with acquired hemophagocytosis Iymphohistiocytosis[J]. Blood, 2008(112): 3552.
21. Henter JI, Samuelsson-Horne A, Arico M, et al. Treatment of hemophagocyte lymphohistiocytosis with HLH-94 immunochemotherapy and Bone marrow transplantation[J]. Blood, 2002, 100(7): 2367-2373.
22. Filipovich AH. Life-threatening hemophagocytic syndromes: current outcomes with hematopoietic stem cell transplantation[J]. Pediatr transplant, 2005, 9(Suppl 7): 87-91.

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