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老年嗜血细胞综合征3例并文献复习

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摘要 噬血细胞综合征(hemophagocytic syndrome,HPS)又称噬血细胞性淋巴组织细胞增多症,是一组以发热,肝脾肿大,全血细胞减少以及骨髓、肝、脾、淋巴结组织发现噬血现象为主要特征的临床综合征.嗜血细胞综合征根据病因分为原发性和获得性,原发性HPS多见于婴幼儿,发病率为1/50 000,2岁以内发病的患者占90%以上,70%~80%于1岁以内发病,8岁以后发病者罕有报道[1].获得性HPS的原因可为感染、肿瘤、免疫介导性疾病等.儿童多由病毒及细菌感染所致,成人除常继发于感染,还可继发于恶性淋巴瘤及免疫相关性疾病等.
作者 王洁妤 李耘
出处 《疑难病杂志》 CAS 2013年第11期887-888,共2页 Chinese Journal of Difficult and Complicated Cases
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参考文献7

  • 1Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis[ J ].Annu Rev Med ,2012,63:233-246.
  • 2Zur Stadt U, Beutel K, Kolberg S, et al. Mutation spectrum in children with primary hcmophagocytic lymphohistiocytosis:molecular and function- al analyses of PRF-1, UNC13D, STX11, and RAB27A [ J ]. Hum Murat, 2006,27 ( 1 ) :62-68.
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二级参考文献21

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