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目的了解噬血细胞综合征的临床特点,治疗及预后。方法分析22例噬血细胞综合征患儿的临床资料。结果临床表现为持续发热,肝脾淋巴结肿大,2系以上外周血细胞减少,肝功异常,凝血障碍,低纤维蛋白血症,高甘油三脂血症,骨髓涂片或淋巴结活检有增多的噬血细胞。结论噬血细胞综合征临床表现凶险,对高度怀疑为噬血细胞综合征的患者,应及早干预治疗,改善预后。
Objective To understand the clinical features, treatment and prognosis of hemophagocytic syndrome. Methods The clinical data of 22 patients with hemophagocytic syndrome were analyzed. Results Clinical manifestations of persistent fever, hepatosplenomegaly, cystic hyperplasia of more than 2 lines, abnormal liver function, coagulopathy, hypofibrinogenemia, hypertriglyceridemia, bone marrow smear or lymph node biopsy increased phagocytosis blood cells. Conclusion Hemophagocytic syndrome clinical manifestations of dangerous, highly suspected hemophagocytic syndrome patients should intervene early treatment and improve prognosis.