儿童噬血细胞综合征26例实验室检查分析

Analysis of laboratory examination in 26 children with hemophagocytic syndrome

目的探讨儿童噬血细胞综合征的临床特点。方法回顾性分析26例感染相关性噬血细胞综合征病的临床及实验室资料。结果感染相关性17例(65.38%),非感染相关性3例(11.54%),原因不明6例(23.08%)。全组病例临床主要特点为持续高热(100.0%)、肝肿大(84.62%)、脾肿大(92.31%);外周血白细胞计数减少(76.92%)、血小板减少(88.46%)、血红蛋白减少(80.77%);骨髓涂片找到噬血细胞(100.0%)。结论儿童感染相关性噬血细胞综合征临床以持续高热、肝肿脾大为主要表现,实验室检查白细胞计数、血红蛋白及血小板中两系或三系减少、低纤维蛋白血症、高脂血症、肝功能、心功能异常及血清铁蛋白升高,骨髓找到噬血细胞等为主要特点。

Objective To investigate the clinical features of children with hemophagocytic syndrome （I-IPS）. Methods The clinical and laboratory data of 26 cases with infection-associated HPS were analyzed retrospectively. Results In all the 26 cases of HPS, 17 cases were infection-associated（65.38% ）, 3 cases were not infection-associated （11.54% ）, 6 cases were unknown causes （23.08%）. The main clinical characteristics of the disease in this group were sustaining fever （100. 00% ）, hepatomegaly （84. 62% ）, splenomegaly （92. 31% ）. Laboratory tests showed decrease of peripheral white blood count （76. 92% ）, of blood platelet （88.46%）, of hemoglobin （80. 77% ）, hemophagocytes in bone marrow slides （ 100. 00% ）. Conclusion The main manifestations of HPS are continuing hyperpyrexia, hepatomegaly and splenomegaly ; the laboratory findings include decrease of white blood count, hemoglobin or blood platelet, hypertriglyceridemia, liver disfunction, elevation of serum ferritin, and the hemophagocytes in bone marrow.