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“噬血细胞性淋巴组织细胞增生症诊疗建议”解读 被引量：7

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摘要 一、前言噬血细胞性淋巴组织细胞增生症（hemophagocyticlymphohistiocytosis，HLH）也称噬血细胞综合征（hemophagocytic syndrome，HPS），目前分为原发性和继发性两大类。其中原发性HLH又可分为两类：一是伴有明显家族遗传倾向者，称为家族性HLH（familial hemophagocyticlymphohistiocytosis，FHL）；二是原发性免疫缺陷病相关性HLH。
作者 汤永民
机构地区 浙江大学医学院附属儿童医院血液肿瘤科
出处 《中华儿科杂志》 CAS CSCD 北大核心 2012年第11期826-828,共3页 Chinese Journal of Pediatrics
关键词 噬血细胞性淋巴组织细胞增生症 原发性免疫缺陷病 噬血细胞综合征 解读 诊疗 遗传倾向 HLH 家族性
分类号 R725.5 [医药卫生—儿科]
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参考文献15

  • 1Farquhar JW, Claireaux AE. Familial haemophagocytic reticulosis. Arch Dis Child, 1952,27:519-525.
  • 2Henter JI, Horne A, Arico M, et al. HLH-2004 : diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer, 2007, 48 : 124-131.
  • 3Jordan MB, Allen CE, Weitzman S, et al. How I treat hemophagocytic lymphohistiocytosis. Blood, 2011, 118 : 4041- 4052.
  • 4] Allen CE, Yu X, Kozinetz CA, et al. Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer,2008,50 : 1227-1235.
  • 5I Gupta A, Weitzman S, Abdelhaleem M. The role of hemophagocytosis in bone marrow aspirates in the diagnosis of hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer, 2008, 50 : 192-194.
  • 6Tang YM, Xu XJ. Advances in hemophagocytic lymphohistiocytosis : pathogenesis, early diagnosis/differential diagnosis, and treatment. Sci World J, 2011, 11 : 697-708.
  • 7Clementi R, Emmi L, Maccario R, et al. Adult onset and atypical presentation of hemophagocytic lymphohistiocytosis in siblings carrying PRF1 mutations. Blood, 2002,100:2266-2267.
  • 8Nagafuji K, Nonami A, Kumano T, et al. Perforin gene mutations in adult-onset hemophagocytic lymphohistiocytosis. Haematologica,2007,92 : 978-981.
  • 9I Antonio G. Tristanol, 2 Macrophage activation syndrome: a frequent but under-diagnosed complication associated with rheumatic diseases. Med Sci Monit, 2008,14: RA27-36.
  • 10Tang Y, Xu X, Song H, et al. Early diagnostic and prognostic significance of a specific Thl/Th2 cytokine panern in children with haemophagocytic syndrome. Br J Haematol,2008,143: 84-91.

同被引文献59

  • 1顾龙君.儿童急性淋巴细胞白血病诊疗建议(第三次修订草案)[J].中华儿科杂志,2006,44(5):392-395. 被引量：472
  • 2Janka GE, Lehmberg K. Hemophagocytic syndromes-an update[J]. Blood Rev, 2014, 28(4): 135-142.
  • 3Dellinger RP, Levy MM, Rhodes A, et al. Surviving sepsis campaign: international guidelines for management of severe sepsis and septic shock: 201213]. Crit Care Med, 2013, 41(2): 580-637.
  • 4Raschke RA, Garcia-Orr R.Hemophagocytic lymphohistiocyto- sis: a potentially underrecognized association with systemic inflammatory response syndrome, severe sepsis, and septic shock in adults[J]. Chest, 2011, 140(4): 933-938.
  • 5Niece JA, Rogers ZR, Ahmad N, et al. Hemophagocytictymphohistiocytosis in Texas: observations on ethnicity and race[J]. Pediatr Blood Cancer, 2010, 54(3): 424-428.
  • 6de Saint Basile G, Mdnaschd G, Fischer A. Molecular mechanisms of biogenesis and exocytosis of cytotoxic granuules[J]. Nat Rev Immunol, 2010, 10(8): 568-579.
  • 7Hall MW, Knatz NL, Vetterly C, et al. Immunoparalysis and nosocomial infection in children with multiple organ dysfunction syndrome[J]. Intensive Care Med, 2011, 37(3): 525-532.
  • 8Allen CE, Yu X, Kozinetz CA, et al. Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis[J]. Pediatr Blood Cancer, 2008, 50(6): 1227-1235.
  • 9Xu XJ, Tang YM, Song H, et al. Diagnostic accuracy of a specific cytokine pattern in hemophagocytic lymphohistiocytosis in children[J]. J Pediatr, 2012,160(6): 984-990.e 1.
  • 10Tang YM, Xu XJ. Advances in hemophagocytic lymphohistiocytosis: pathogenesis, early diagnosis/differential diagnosis, and treatment[J]. Sci World J, 2011, 11 : 697-708.

引证文献7

二级引证文献8

中华儿科杂志
2012年 第11期

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