摘要
噬血细胞淋巴组织细胞增生症（hemophagocytic lymphohistiocytosis，HLH），或称噬血细胞综合征（hemophagocytic syndrome），是一种单核巨噬系统过度反应性疾病，其临床特征为发热、肝脾和（或）淋巴结肿大、全血细胞减少、肝功能异常和凝血障碍，组织学特征为组织细胞或巨噬细胞的过度增生与活化。近年来的研究深化了对该病的发病机制及治疗的认识，现综述如下。
出处
《实用医学杂志》
CAS
2007年第21期3461-3464,共4页
The Journal of Practical Medicine
参考文献24
-
1Ueda I, Ishii E, Morimoto A, et al. Correlation between phenotypic heterogeneity and gene mutational characteristics in familial hemophagocytic lymphohistiocyt-osis (FHL) [J]. Pediatr Blood Cancer, 2006,46 (4) : 482-488.
-
2Zur S U, Beutel K, Kolberg S, et al. Mutation spectrum in children with primary hemophagocytic lymphohistiocytosis : molecular and functional analyses of PRF1, UNC13D, STXll, and RAB27A [ J ]. Hum Mutat, 2006,27 ( 1 ) : 62-68.
-
3Takada H, Ohga S, Mizuno Y,et al. Increased IL-16 levels in hemophagocytic lymphohistiocytosis [J ]. J Pediatr Hematol Oncol, 2004,26(9) : 567-573.
-
4Takada H, Nomura A, Ohga S, et al. Interleukin- 18 in hemophagocytic lymphohistiocy-tosis. Leuk Lymphoma [J]. 2001,42(1-2) :21-8.
-
5Billiau A D, Roskams T, DammeLombaerts R, et al. Macrophage activation syndrome:characteristic findings on liver biopsy illustrating the key role of activated, IFN-gammaproducinglymphocytes and IL-6- and TNFalpha-produeing macrophages [J ]. Blood,2005, 105(4) : 1648-1651.
-
6Henter J I. Biology and treatment of familial hemophagoeytie lymphohistiocytosis: importantce of perforinin lymphocytemediated cytotoxicity and triggering of apoptosis [ J]. Med Pediatr Oncol, 2002, 38(5) :305-309.
-
7Stepp S E, Dufourcq-Lagelouse R,le Deist F, et al. Perforin gene defects In familial hemophog-ocytic lymphohistiocytosis [J]. Science, 1999,286 (5446) : 1957-1959.
-
8Catalfamo M, Henkart P A. Perforin and the granule exocytosis cytotoxieity pathway [J]. Curr Opin Immunol,2003,15(5): 522-527.
-
9Raja S M, Metkar S, Froehlich J. Cytotoxic granulemediated apoptosis: unraveling the complex mechanism [J]. Curr Opln Immunol, 2003,15 (5) : 528-532.
-
10Russell J H, Ley T J, Lymphocyte-mediated cytotoxicity [J]. Annu Rev I mmunol, 2002,20 : 323-370.
同被引文献16
-
1张楠,郭树彬.噬血细胞综合征[J].中国急救复苏与灾害医学杂志,2006,1(4):188-190. 被引量：6
-
2曹国秀,刘芳,杜娟,刘兰英,李芳.化疗药物静脉注射和静脉输注对血管损伤的观察[J].护理研究（中旬版）,2005,19(7):1281-1282. 被引量：9
-
3罗绍凯,洪文德,李娟.临床血液病学[M].北京:科学技术出版社,2002:240.
-
4Henter JI, Horne AC, Arico M, et al. HLH - 2004 : diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis [ J ]. Pediatr Blood Cancer,2007,48 ( 2 ) : 124 - 131.
-
5De Saint Basi|e G,Fischer A. The role of cytotoxicity in lymphocyte homeostasis[ J ]. Curr Opin Immunol,2001,13 (5) :549 - 554.
-
6Tsuda H. Hemophagocytic syndrome (I'IPS) in children and adults [J]. Int J Hematol,1997,65(3) :215 -226.
-
7Janka GE. Hemophagocytic syndromes [ J ]. Blood Rev, 2007,21 (5) :245 - 253.
-
8Verbsky JW, Grossman WJ. Hemophagocytic lymphohistiocytosis: diagnosis, patophysiology, treatment, and future perspectives [ J ]. Ann Med, 2006, 38(1) :20-31.
-
9Henter J, Home A, Arico M, et al. HLH-2004 : Diagnosticand therapeutic guidelines for hemophagocytic lymphohistiocytosis [ J ]. Pediatr Blood Cancer, 2007, 48 ( 2 ) : 124-131.
-
10Shinsaku I, Kikuko K, Rika S, et al. Treatment of Epstein-Barr vi- res-associated hemophagocytic lymphohistiocytosis (EBV-HLI-I) in young adults: a report from the HLH study center[J]. Med Pediatr Oncol, 2003,41 (2) :103-109.
引证文献3
-
1王茜,陈军.肿瘤相关性噬血细胞综合征1例[J].现代中西医结合杂志,2012,21(27):3048-3049. 被引量：1
-
2任彩云,王闰平,张蕾,方嫦娥.足叶乙苷治疗噬血细胞综合征的护理[J].中国药业,2012,21(21):95-96.
-
3李洪丽,王焱,毕可红,朱传升.弥漫大B细胞淋巴瘤继发噬血细胞综合征1例[J].山东医药,2014,54(1):106-107.
-
1楼玲莉.噬血细胞综合征研究进展[J].青海医药杂志,2005,35(4):63-64.
-
2付功丽,刘芳.肿瘤相关性噬血细胞综合征的诊断及治疗[J].临床血液学杂志,2016,29(3):357-362. 被引量：4
-
3曲延章,张淑英,韩树林,周虹.成人噬血细胞综合征40例临床分析[J].中国实用医药,2010,5(20):105-106. 被引量：4
-
4卢根,申昆玲.儿童噬血细胞淋巴组织细胞增生症[J].中国循证儿科杂志,2008,3(2):143-150. 被引量：6
-
5张景荣,梁筱灵,靳蓉,卢根.HLH-2004方案诊治儿童噬血细胞综合征临床分析[J].中国当代儿科杂志,2013,15(8):686-688. 被引量：12
-
6李芳秋,陆瑾,杨敏.噬血细胞综合征发病机制及实验诊断[J].临床检验杂志,2004,22(1):53-54. 被引量：22
-
7申淑珍,崔丽娟,宋国庆.噬血细胞性淋巴组织细胞增生症6例临床分析[J].宁夏医学杂志,2008,30(7):613-614.
-
8郝燕.重症监护病房噬血细胞淋巴组织细胞增生症临床分析[J].中国社区医师,2016,32(12):71-72.
-
9常勇,陈红兵,徐飞,曹波,刘雪梅,赵呈元.噬血细胞淋巴组织细胞增生症患儿外周血淋巴细胞亚群的变化及意义[J].广东医学,2014,35(20):3220-3222.
-
10蔡桂兰,王旖旎,陈葵,韩燕飞,张拥波.成人噬血细胞淋巴组织细胞增生症的中枢神经系统病变临床分析[J].临床和实验医学杂志,2015,14(10):791-794. 被引量：2
