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噬血细胞性淋巴组织细胞增多症发病机制研究进展 被引量：1

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摘要 噬血细胞性淋巴组织细胞增多症（HLH），又称噬血细胞综合征（HPS），1952年首次报道，具有多脏器受损、病情进展迅猛、延误诊治病死率高的临床特点。该病病因病机错综复杂，早期症状不典型，诊断非常困难，且病情进展迅速，许多患儿因早期不能明确诊断和及时治疗导致死亡。现对HLH分子生物学基础及发病机制、临床表现、诊断、鉴别诊断及治疗进行综述。
作者 任一帆 贾国存
机构地区 郑州市儿童医院血液科
出处 《中国小儿血液与肿瘤杂志》 CAS 2014年第1期47-50,共4页 Journal of China Pediatric Blood and Cancer
关键词 淋巴组织细胞增多症 噬血细胞性 发病机制 噬血细胞综合征 分子生物学基础 鉴别诊断 病情进展 多脏器受损
分类号 R725.5 [医药卫生—儿科]
  • 相关文献

参考文献25

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  • 3Marcenaro S,Gallo F,Martini S. Analysis of natural killer-cell function in familial hemophagocytic lymphohistiocytosis (FHL):defective CD107a surface expression heralds Muncl13-4 defect and discriminates between genetic subtypes of the disease[J].Blood,2006.2316-2323.
  • 4Yoon HS,Kim HJ,Yoo KH. UNC13D is the predominant causative gene with recurrent splicing mutations in Korean patients with familial hemophagocytic lymphohistiocytosis[J].Haematologica,2010.622-626.
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  • 6陈华,王昭,王旖旎,王晶,石吴林.原发性噬血细胞综合征一例报告附文献复习[J].中华血液学杂志,2010,31(11):779-780. 被引量：7
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  • 8黄志卓(综述),谢正德(审校),申昆玲(审校).原发性噬血细胞性淋巴组织细胞增生症遗传学研究进展[J].国际儿科学杂志,2011,38(3):287-290. 被引量：4
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二级参考文献20

  • 1Menasche G, Feldmann J, Fischer A, et al. Primary hemophagocytic syndromes point to a direct link between lymphocyte cytotoxicity and homeostasis[J ]. Immunol Rev, 2005,203 : 165 - 179.
  • 2Ohadi M, Lalloz MR, Sham P, et al. Localization of a gene for familial hemophagocytic lymphohistiocytosis at chromosome 9q21.3 22 by homozygosity mapping[ J ]. Am J Hum Genet, 1999, 64 ( 1 ) : 165-171.
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  • 7Feldmann J, Callebaut I, Raposo G, et al. Muncl3-4 is essential for cytolytic granules fusion and is mutated in a form of familial hemophagocytic lymphohistiocytosis ( FHL3 ) [ J ]. Cell, 2003, 115(4) :461 - 473.
  • 8Yamamoto K, Ishii E, Sako M, et al. Identification of novel MUNC 13-4 mutations in familial haemophagocytic lymphohistiocytosis and functional analysis of MUNC13-4 deficient cytotoxic T lymphocytes[J],J Med Genet,2004,41(10):763-776.
  • 9Zur Stadt U, Schmidt S, Kasper B, et al. Linkage of familial hemophagocytic lymphohistiocytosis (FHL) type - 4 to chromosomc 6q24 and identification of mutations in syntaxin 11 [J ].Hum Mol Genet ,2005,14(6) :827 - 834.
  • 10Yamamoto K, Ishii E, Horiuchi H, et al. Mutations of syntaxin 11 and SNAP23 genes as causes of familial hemophagocytic lymphohistiocytosis were not found in Japanese people[J ]. J Hum Genet, 2005,50( 11 ) :600-603.

共引文献26

同被引文献30

  • 1Han AR, Lee HR, Park BB, et al. Lymphoma-associated hemophagocytic syndrome: clinical features and treatment outcome[J]. Ann Hematol, 2007, 86(7): 493-498.
  • 2Xie W, Hu K, Xu F, et al. Clinical analysis and prognostic significance of lymphoma-associated hemophagocytosis in peripheral T cell lymphoma[J]. Ann Hematol, 2013, 92(4): 481-486.
  • 3Takahashi N, Miura I, Chubachi A, et al. A clinicopathological study of 20 patients with T/natural killer (NK)-cell lymphoma-associated hemophagocytic syndrome with special reference to nasal and nasal- type NK/T-cell lymphoma[J]. Int J Hematol, 2001, 74(3): 303-308.
  • 4Henter JI, Horne A, Aric6 M, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagoc)rtic lymphohistiocytosis[J]. Pediatr Blood Cancer, 2007, 48(2): 124-131.
  • 5Choi YL, Park JH, Kim WS, et al. Aggressive NK-cell leukaemia associated with reactive haemophagocytic syndrome[J]. Clin Exp Dermatol, 2006, 31(1): 83-85.
  • 6Chuang HC, Lay JD, Hsieh WC, et al. Pathogenesis and mechanism of disease progression from hemophagocytic lymphohistiocytosis to Epstein-Barr virus-associated T-cell lymphoma: nuclear factor- kappa B pathway as a potential therapeutic target[J]. Cancer Sci, 2007, 98(9): 1281-1287.
  • 7Takai K, Nikkuni K, Sanada M, et al. EB virus-associated peripheral T cell lymphoma presenting with hemophagocytic syndrome and hepatic cell necrosis[J]. Rinsho Ketsueki, 2003, 44(7): 462-467.
  • 8Hart L, Zhang M, Li L, et al. Natural killer/T-cell lymphoma- associated hemophagocytic syndrome: a case report[J]. Oncol Lett, 2014, 8(2): 886-890.
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  • 10Sano H, Kobayashi R, Tanaka J, et al. Risk factor analysis of non- Hodgkin lymphoma-associated haemophagocytic syndromes: a multicentre study[J]. Br J Haematol, 2014, 165(6): 786-792.

引证文献1

二级引证文献2

中国小儿血液与肿瘤杂志
2014年 第1期

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