【摘要】 目的分析儿童噬血细胞性淋巴组织细胞增生症的临床特征及治疗效果。方法针对35例初诊为儿童噬血细胞性淋巴组织细胞增生症患儿的病因、临床特点及治疗效果进行回顾性分析。结果本组患儿临床特点以发热(100%)、脾肿大(86%)及肝肿大(80%)最常见;实验室检查以铁蛋白≥500μg/L(100%)、两系或三系血细胞减少(83%)及NK细胞活性降低29例(83%)最为常见;病因以EBV感染(30%)最常见;缓解组与未缓解组肝肿大、脾肿大、WBC、Hb、PLT、NEU、TG、FIB、NK活性、AST、ALT无统计学意义,缓解组总T细胞百分比高于未缓解组,差异有统计学意义(P=0.043)。结论儿童噬血细胞性淋巴组织细胞增生症病因复杂,总T细胞百分比可能是预后的影响因素。更多还原

【Abstract】 Objective to analyze clinical features and curative effect of hemophagocytic lymphohistiocytosis children. Methods review and analyze etiology, clinical features and curative effect of 35 cases hemophagocytic lymphohistiocytosis children. Results the most common clinical features include fever(100%), splenomegaly(86%)and hepatomegaly(80%) in the group. Laboratory tests show the most common situation includes ferritin ≥500μg/L)(100%), two or three lineage hematocytopenia(83%) and 29 cases of NK cytoactive decrease(83%); EBV infection(40%)was the most common etiology. Hepatomegaly, splenomegaly, WBC, Hb, PLT, NEU, TG, FIB, NK activity, AST, ALT showed no statistical significance between relief group and non-relief group. Total T cells percentage in relief group was higher than non-relief group, difference showed statistical significance,(P=0.043). Conclusion hemophagocytic lymphohistiocytosis of children has complicated etiology, and total T cells percentage may be one of prognostic influencing factors.更多还原