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噬血细胞综合征一例 被引量：1

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摘要 患者男，2岁，因反复发热10d人住我院小儿科，当时无咽痛、咳嗽、咳痰，无腹痛、腹泻，查血常规：
作者 谭振清 罗自勉 周新伏 尹亚飞 刘利华
机构地区 [
出处 《白血病．淋巴瘤》 CAS 2012年第9期572-573,共2页 Journal of Leukemia & Lymphoma
关键词 淋巴组织细胞增多症 嗜血细胞性 药物疗法 联合
分类号 R733 [医药卫生—肿瘤]
  • 相关文献

参考文献11

  • 1Henter JI, Home A, Arico M, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer, 2007, 48: 124-131.
  • 2Henter JI, Elinder G, Soder O, et al. Hypercytokinemia in familial hemophagocytic lymphohistiocytosis. Blood, 1991, 78: 2918-2922.
  • 3Grunebaum E, Roifman CM. Gene abnormalities in patients with hernophagocytic lymphohistiocytosis. Isr Med Assoc J, 2002, 4: 366- 369.
  • 4Stepp SE, Dufourcq-Lagelouse R, Lc Deist F, et al. Perforin gene defects in familial hemophagocytic lymphohistiocytosis. Science, 1999, 286: 1957-1959.
  • 5Feldmann J, Callebant I, Raposo G, et al. Muncl3-4 is essential for cytolytic granules fusion and is mutated in a form of familial hemophagocytic lymphohistiocytosis (FHL3). Cell, 2003, 115: 461- 473.
  • 6Stadt U, Schmidt S, Kasper B, et al. Linkage of familial hemophagocytic lymphohistiocytosis (FHL) type-d- to chromosome 6q24 and identification of mutations in syntaxin 11. Hum Mol Genet, 2005, 14: 827-834.
  • 7Cote M, Menager MM, Burgess A, et al. Munc18-2 deficiency causesfamilial hemophagoeytic lymphohistiocytosis type 5 and impairs cytotoxic granule exocytosis in patient NK cells. J Clin Invest, 2009, 119: 3765-3773.
  • 8Stadt U, Rohr J, Seifert W, et al. Familial hemophagocytic lymphohistiocytosis type 5 (FHL-5)is caused by mutations in Muncl 8- 2 and impaired binding to syntaxin 11. Am J Hum Genet, 2009, 85: 482-492.
  • 9Bryceson YT, Rudd E, Zheng C, et al. Defective cytotoxic lymphocyte degranulation in syntaxin-ll deficient familial hemophagocytic lymphohistiocytosis 4 (FHIA) patients. Blood, 2007, 110:1906-1915.
  • 10Meeths M, Entesarian M, A1-Herz W, et al. Spectrum of clinical presentations in familial hemophagocytic lymphohistiocytosis type 5 patients with mutations in STXBP2. Blood, 2010, 116: 2635-2643.

二级参考文献4

  • 1Henter JI, Home A, Arico M, et al. HLH-2004 : Diagnostic and therapeutic guidelines for hemophagucytie lymphohistiocytosis. Pediatr Blood Cancer,2007,48 (2) : 124-131.
  • 2Henter JI, Elinder G, OstA. the FHL Study Group of the Histiocyte Society. Diagnostic guidelines for hemophagocytic lymphohistiocytosis. Semin Oncol, 1991,18:29-33.
  • 3Abha Gupta, Pascal Tyrrell, Rahim Valani, et al. The Role of the Initial Bone Marrow Aspirate in the Diagnosis of Hemophagocytic Lymphohistiocytosis. Pediatr Blood Cancer,2008,51:402 -404.
  • 4Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis. Eur J Pediatr,2007 ,166 :95-109.

共引文献4

同被引文献23

  • 1Jaiika GE. Hemophagocytic syndromes. Blood Rev, 2007, 21: 245-253.
  • 2Szyper-Kravitz M. The hemophagocytic syndrome / macrophageactivation syndrome: a final common pathway of a nytokinestorm. IsrMed Assoc J, 2009,11 : 633-634.
  • 3Risdall RJ, McKenna RW, Nesbit ME, et al. Virus-associatedhemophagocytic syndrome: a benign histiocytic proliferation distinctfrom malignant histiocytosis. Cancer, 1979, 44: 993.
  • 4Perez N, Virelizier JL, Arenzana-Seisdedos F, et al. Impaired naturalkiller activity in lymphohistiocytosis syndrome. J Pediatr, 1984, 104:569-573.
  • 5Eife R, Janka GE, Belohradsky BH, et al. Natural killer cell functionand interferon production in familial hemophagocyticlymphohistiocytosis. Pediatr Hematol Oncol, 1989, 6:265-272.
  • 6Arico M, Janka G, Fischer A, et al. Hemophagocyticlymphohistiocytosisrdiagnosis,treatment and prognostic factorsrreportof 122 children from the international registry. Leukemia, 1996, 10:197-203.
  • 7Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis.Eur J Pediatr, 2007, 166: 95-109.
  • 8Katano H, Cohen JI. Perforin and lymphohistiocytio proliferativedisorders. Br J Haematol, 2005, 128: 739-750.
  • 9Feldmann J,Callebaut I, Raposo G, et al. Munc 13-4 is essential forcytolytic granules fusion and is mutated in a form of familialhemophagocytic lymphohistiocytosis (FHL3). Cell, 2003, 115: 461-473.
  • 10Larroche C, Mouthon L. Pathogenesis of hemophagocytic syndrome(HPS). Autoimmun Rev, 2004, 3: 69-65.

引证文献1

二级引证文献2

白血病．淋巴瘤
2012年 第9期

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