噬血细胞综合征临床和治疗

Clinic and therapy of hemophagocytic syndrome

目的 了解噬血细胞综合征 (HS)的临床特点和治疗。方法 分析 1 4例HS患儿临床表现、实验室检查、治疗及预后。结果 所有患儿有不同程度的肝功能损害 ,全血细胞减少 ,凝血功能障碍 ,低纤维蛋白血症 ,高甘油三脂血症 ,高细胞因子血症 ,铁蛋白增高 ,细胞免疫功能异常 ,骨髓片找到噬血细胞。结论 临床高度怀疑噬血细胞综合征应及早干预治疗。

Objective To investigate the clinical features and therapy of hemophagocytic syndrome(HS). Methods The clinical manifestation, laboratory investigation, therapy and prognosis in 14 patients with HS were analysised. Results In all of the patients, there were defferent degree in liver dysfunction, pancytopenia, coagulation abnormalities, hypofibrinogenemia, hypertriglyceridemia, hypercytokinemia, hyperferritinemia, abnormalities of celluar immunity, hemophagocytic histiocytes were observed in the bone marrow. Conclusion Early therapy should be given if hemophagocytic syndrome was highly suspected.