本文關(guān)鍵詞：205例繼發(fā)性噬血細(xì)胞淋巴組織細(xì)胞增生癥患者的臨床特征及預(yù)后相關(guān)因素分析　出處：《浙江大學(xué)》2017年碩士論文　論文類型：學(xué)位論文

  更多相關(guān)文章： 噬血細(xì)胞淋巴組織細(xì)胞增生癥 病因 總生存時(shí)間 預(yù)后

【摘要】：背景:噬血細(xì)胞淋巴組織細(xì)胞增生癥(Hemophagocytic lymphohistiocytosis,HLH)是一類免疫介導(dǎo)并威脅生命的非常見的臨床綜合征,因其常在成人多種疾病中伴隨出現(xiàn),故其診斷極具挑戰(zhàn)性。方法:我們對(duì)浙江大學(xué)醫(yī)學(xué)院附屬第一醫(yī)院在2011年1月至2015年12月期間符合HLH-2004診斷標(biāo)準(zhǔn)的205例資料完整的成人HLH患者的臨床表現(xiàn)、實(shí)驗(yàn)室檢查、治療方案及臨床轉(zhuǎn)歸等資料進(jìn)行回顧性綜合分析。結(jié)果:205例患者中位年齡為52歲(范圍18-90歲),其中123例(60.0%)為男性。超過95%的患者有發(fā)熱、高鐵蛋白血癥和乳酸脫氫酶升高。HLH的病因包括惡性腫瘤(119例,58.0%),感染(83例,40.5%),自身免疫性疾病(14例,6.8%)和未知原因(14例,6.8%)�；颊咧形簧鏁r(shí)間為55天,老年人(≥60歲)的預(yù)后較年輕人(60歲)差(中位生存時(shí)間24天vs.159天,P0.001)。在多因素方差分析中,血小板計(jì)數(shù)40×109/L(HR = 2.273;95%CI:1.246-5.948;P = 0.012)、凝血酶原時(shí)間延長(zhǎng)3秒(HR = 2.008;95%CI:1.190-3.390;P=0.009)、腺苷酸脫氨酶89 U/L(HR = 1.706;95%CI:1.015-2.865;P = 0.044)及低密度脂蛋白3.29 mmol/L(HR = 0.196;95%CI:0.048-0.806;P = 0.024)提示不良預(yù)后。結(jié)論:成人HLH的生存時(shí)間均極為短暫,因而提高對(duì)這一綜合征的認(rèn)識(shí),完善診斷標(biāo)準(zhǔn)及提高治療有效率均具有重要意義。
[Abstract]:Background: hemophagocytic lymphohistiocytosis in hemophagocytic histiocytosis. HLH is a class of immune mediated and life-threatening clinical syndrome, because it is often accompanied by a variety of adult diseases. Therefore, its diagnosis is very challenging. Methods:. The clinical table of 205 adult HLH patients who met the diagnostic criteria of HLH-2004 between January 2011 and December 2015 in the first affiliated Hospital of Zhejiang University School of Medicine. Right now. Results the median age of 205 patients was 52 years (range 18-90 years). More than 95% patients had fever, and the causes of high ferricinemia and lactate dehydrogenase (LDH) were 58.0% of malignant tumors. The median survival time of the patients was 55 days. The prognosis of the elderly (鈮，

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