發(fā)布時間：2018-02-09 01:27

  本文關鍵詞： 嗜血細胞性淋巴組織細胞增生癥 兒童 危險因素 預后　出處：《臨床血液學雜志》2015年06期 　論文類型：期刊論文

【摘要】：目的:通過分析嗜血細胞性淋巴組織細胞增生癥(HLH)患兒的臨床表現、診斷標準、治療以及預后情況,探討影響HLH預后的危險因素。方法:回顧性分析65例HLH患兒的臨床特征、實驗室檢查結果、治療方案及預后。結果:65例HLH患兒中,男女之比為1.71∶1.00,發(fā)病中位年齡26個月。病因以EB病毒相關性HLH最為常見,共35例(53.85%),風濕免疫性疾病相關HLH 2例(3.08%),原發(fā)性HLH 2例(3.08%)。臨床表現以發(fā)熱65例(100%),肝臟腫大63例(96.92%),脾臟腫大54例(83.08%)最為常見。實驗室檢查以起病時肝功能異常最為明顯,鐵蛋白升高65例(100%),外周血兩系以上細胞減少55例(84.62%),纖維蛋白原降低55例(84.62%),高甘油三酯血癥(TG≥3.0mmol/L)47例(72.31%),骨穿發(fā)現嗜血現象39例(60.94%)。單因素分析顯示,出血傾向、低白蛋白血癥及總膽紅素升高是影響HLH預后的危險因素;多因素Logistic分析顯示,出血傾向及低白蛋白血癥是影響HLH預后的獨立危險因素。結論:HLH病因復雜,臨床表現和實驗室檢查異常多樣,預后兇險,早期診斷和規(guī)范治療十分重要。出血傾向和低白蛋白血癥是影響HLH預后的危險因素。
[Abstract]:Objective: to investigate the risk factors influencing the prognosis of HLH by analyzing the clinical manifestations, diagnostic criteria, treatment and prognosis of hemophilic lymphohistiocytosis. Methods: the clinical features of 65 cases of HLH were retrospectively analyzed. Results among 65 children with HLH, the ratio of male to female was 1.71 to 1.00, and the median age was 26 months. Epstein-Barr virus-associated HLH was the most common etiology. A total of 35 cases (53.85%), 2 cases of HLH associated with rheumatic immune diseases (3.08), 2 cases of primary HLH (3.08%), 65 cases of fever (65 cases), 63 cases of hepatomegaly (96.92%), 54 cases of splenomegaly (83.08%) were the most common clinical manifestations. There were 65 cases of elevated ferritin, 55 cases of reduction of peripheral blood cells above two lineages (84.62%), 55 cases of fibrinogen reduction (84.62%), 47 cases of hypertriglyceridemia (TG 鈮，

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