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噬血细胞综合征发病机制的研究进展 被引量：3

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摘要 噬血细胞综合征（hemophagocyti csyndromes,HPS）,又称噬血细胞性淋巴组织细胞增多症（hemophagocyti clymphohistocyto-sis,HLH）,是由多种致病因素引起的淋巴细胞和组织细胞过度增殖、活化,产生大量炎性因子,从而引起的一种可危及生命的过度炎性反应[1]。HPS可分为原发性HPS及获得性HPS。原发性HPS有部分存在穿孔素基因突变或其他遗传免疫的缺陷。
作者 林榕榕 王昭
机构地区 首都医科大学附属北京友谊医院血液科
出处 《河北医药》 CAS 2011年第9期1389-1390,共2页 Hebei Medical Journal
关键词 噬血细胞综合征 家庭性噬血细胞综合征 免疫缺陷综合征
分类号 R593.3 [医药卫生—内科学]
  • 相关文献

参考文献18

  • 1Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis. Eur J Pediatr,2007 ,166 :95-109.
  • 2王旖旎,王昭,吴林,岑溪南,李渤涛,宁丰,段学章,李娟.多中心72例噬血细胞综合征诊疗分析[J].中华血液学杂志,2009,30(12):793-798. 被引量：98
  • 3Henter Jl, Home AC, Egeler RM, et al. HLH-2004 : Diagnostic and thempeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer,2007,48:124-131.
  • 4王昭,陈皙,吴林,田莉萍,王晶石.噬血细胞现象在诊断噬血细胞性淋巴组织细胞增多症中意义的探讨[J].中国实验血液学杂志,2009,17(4):1064-1066. 被引量：18
  • 5Filipovich AH. Hemophagocytic lymphohistiocytosis (HLH) and related disorders. Hematology Am Soc Hematol Eudc Program,2009,127-131.
  • 6Stepp SE, Dufourcq-Lagelouse R, Le Deist F, et al. Perforin gene defects in familial hemophagocytic lymphohistiocytosis. Science,286:1957-1959.
  • 7Feldmann J, Callwbaut I, Raposo G, et al. Munc 13-4 is essential for cytolytic granules fusion and is mutated in a form of familial heraophagocytic lymphohistiocytosis ( FHL3 ). Cell ,2003,115:461-473.
  • 8zur Stadt U,Schmidt S,Kasper B, et al. Linkage of familial hemophagocytic lymphohistiocytosis(FHL) type-4 to chromosome 6q24 and identification of mutations in syntaxin 11. Hum Mol Genet,2005,14:827-834.
  • 9Ameson LN, Brickshawana A, Segovis CM, et al. Cutting edge: syntaxin 11 regulates lymphocyte-mediated secrdtion and cytotoxicity. J Immunol, 2007,179 : 3397 -3401.
  • 10zur Stadt U, Rohr J, Seifert W, et al. Familial hemophagoeytic lymphohistiocytosis type 5 ( FHL-5 ) is caused by mutations in Munc18-2 and impaired binding to syntaxin 11. Am J Hum C, enet,2009 ,g5:482-492.

二级参考文献14

  • 1Janka GE. Familial and acquired hemophagocytie lymphohistiocytosis. Eur J Pediatr, 2007; 166:95-109.
  • 2Janka GE, Schneider EM. Modem management of children with haemophagocytic lymphohistiocytosis. Br J Haematol, 2004; 124: 4- 14.
  • 3Henter JI, Home AC, Arico M, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer, 2007 ; 48 : 124 - 131.
  • 4Kontopoulou T, Tsaousis G, Vaidakis E, et al. Hemophagoeytic syndrome in association with visceral lcishmaniasis. Am J Med, 2002; 113 : 439 -440.
  • 5Gupta A, Tyrrell P, Valani R, et al. The role of the initial bone marrow aspirate in the diagnosis of hernophagocytic lymphohistiocytosis. Pediatr Blood Cancer, 2008 ; 51 : 402 - 404.
  • 6James WV, William JG. Hemophagocytic lvmphohistioeytosis diagnosis, pathophysiology, treatment, and future perspectives. Ann Med, 2006, 38 : 20-31.
  • 7Hcnter JI, Home A, Arico M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer, 2007, 48:124-131.
  • 8Janka GE. Familial and acquired hemophagocytie lymphohistiocylosis. Eur J Pedialr, 2007, 166: 95-109.
  • 9Teng CL, Hwang GY, Lee B J, et al. Pregnancy-induced hemophagocytic lymphohistiocytosis combined with autoimmune hemolytic anemia. J Chin Med Assoc, 2009, 72: 156-159.
  • 10Allen CE, Yu X, Kozinetz CA, et al. Highly elevated ferritin levels and the diagnosis of hemophagocytie lymphohistiocytosis. Pediatr Blood Cancer, 2008, 50: 1227-1235.

共引文献106

同被引文献34

  • 1高静,李昕权.肝移植围手术期的血液学异常[J].国外医学（移植与血液净化分册）,2005,3(3):14-16. 被引量：1
  • 2谢静,张大伟,杨双,吴敏媛.儿童噬血细胞性淋巴组织增生症28例临床研究[J].中国当代儿科杂志,2006,8(4):345-347. 被引量：4
  • 3王棠海.血液与血液检验学[M].北京:学苑出版社,1990:164-165.
  • 4Henter JI,Horne A,Arico M,Egeler RM,Filipovich AH,Imashuku S,et al.HLH-2004:Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis[J].Pediatr Blood Cancer,2007,48(2):124-131.
  • 5Malloy CA,Polinski C,Alkan S,Manera R,Challapalli M.Hemophagocytic lymphohistiocytosis presenting with nonimmune hydrops fetalis[J].Perinatol,2004,24(7):458-460.
  • 6Ishii E,Ohga S,Imashuku S,Yasukawa M,Tsuda H,Miura I,et al.Nationwide survey of hemophagocytic lymphohistiocytosis in Japan[J].Int J Hematol,2007,86(1):58-65.
  • 7Janka GE.Familial and acquired hemophagocytic lymphohistiocitosis[J].Eur J Pediatr,2007,166(2):95-109.
  • 8Filipovich AH.Hemophagocytic lymphohistiocytosis(HLH)and related disorders[J].Hematol Am Soc Hematol Eudc Program,2009:127-131.
  • 9Imashuku S.Clinical features and treatment strategies of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis[J].Crit Rev Oncol Hematol,2002,44(3):259-272.
  • 10Fukaya S,Yasuda S,Hashimoto T,Oku K,Kataoka H,Horita T,et al.Clinical features of haemophagocytic syndrome in patients with systemic antoimmune disease:analysis of30cases[J].Rheumatology(Oxford),2008,47(11):1686-1691.

引证文献3

二级引证文献14

河北医药
2011年 第9期

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