噬血細胞綜合征的臨床研究進展_《華西醫學》

作者：

劉瀟瀟 ,  練穎

成都市第一人民醫院血液風濕科（成都，610010）;

關鍵詞：

噬血細胞綜合征穿孔素細胞因子 HLH-2004治療方案

DOI：

CNKI: 51-1356/R.20120214.1340.011

視頻：

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噬血細胞綜合征是由淋巴細胞、單核細胞和吞噬細胞系統異常激活、增殖所引起，由此導致的高細胞因子血癥及繼起的過度炎癥反應，以致骨髓或者淋巴組織、器官中出現異常增多的組織細胞，并伴有吞噬自身血細胞行為為特征的綜合征。其病因多樣、臨床表現復雜，如不及時治療，患者可很快死于感染及多器官衰竭。現將近年來對噬血細胞綜合征的臨床研究進行綜述。

引用本文： 劉瀟瀟,練穎. 噬血細胞綜合征的臨床研究進展. 華西醫學, 2012, 27(2): 203-205. doi: CNKI: 51-1356/R.20120214.1340.011 復制

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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.
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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.	張炯, 鄒和建. 自身免疫性疾病并發反應性噬血細胞綜合征2例[J]. 內科理論與實踐, 2008, 3(3): 190-192.
10.	皇甫春榮, 付紅敏. EB病毒相關性噬血細胞綜合征的臨床特征及外周血淋巴細胞亞群變化[J]. 中國小兒急救醫學, 2010, 17(4): 330-332.
11.	Janka G. Hemophagucytie syndromes[J]. Blood Rev, 2007, 21(5): 245-253.
12.	王學文. 噬血細胞綜合征的研究進展[J]. 臨床腫瘤學雜志, 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.	黃道連, 鞠文東, 付四毛, 等. 噬血細胞綜合征早期實驗室指標改變研究[J]. 實驗與檢驗醫學, 2008, 26(6): 598-599.
17.	李芳秋. 噬血細胞綜合征發病機制及實驗診斷[J]. 臨床檢驗雜志, 2004, 22(1): 532-533.
18.	方涵, 謝軍軍, 王劍超. 噬血細胞綜合征患者血清LDH和β 2 -MG檢測的臨床意義[J]. 檢驗醫學, 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.

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. 張炯, 鄒和建. 自身免疫性疾病并發反應性噬血細胞綜合征2例[J]. 內科理論與實踐, 2008, 3(3): 190-192.
10. 皇甫春榮, 付紅敏. EB病毒相關性噬血細胞綜合征的臨床特征及外周血淋巴細胞亞群變化[J]. 中國小兒急救醫學, 2010, 17(4): 330-332.
11. Janka G. Hemophagucytie syndromes[J]. Blood Rev, 2007, 21(5): 245-253.
12. 王學文. 噬血細胞綜合征的研究進展[J]. 臨床腫瘤學雜志, 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. 黃道連, 鞠文東, 付四毛, 等. 噬血細胞綜合征早期實驗室指標改變研究[J]. 實驗與檢驗醫學, 2008, 26(6): 598-599.
17. 李芳秋. 噬血細胞綜合征發病機制及實驗診斷[J]. 臨床檢驗雜志, 2004, 22(1): 532-533.
18. 方涵, 謝軍軍, 王劍超. 噬血細胞綜合征患者血清LDH和β 2 -MG檢測的臨床意義[J]. 檢驗醫學, 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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噬血細胞綜合征的臨床研究進展

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《華西醫學》

噬血細胞綜合征的臨床研究進展

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