儿童噬血细胞综合征的诊断和治疗

目的:提高对儿童噬血细胞综合征(hemophargocytic syndrome,HPS)的认识。方法:回顾性分析24例HPS患者的临床资料。结果:24例HPS患者有15例与感染相关,1例家族性,1例系统性红斑狼疮所致,7例病因未明;临床表现持续性高热24例(100%),肝和(或)脾大24例(100%),血常规二系或三系减少24例(100%),高甘油三酯血症12例,10例患者测血清铁蛋白有9例明显增高,凝血功能异常者18例,丙氨酸转氨酶增高21例,24例骨髓均发现组织细胞增多,有噬血组织细胞,并可见明显吞噬血细胞现象,组织细胞形态基本正常。13例加用糖皮质激素,18例给大剂量人血丙种球蛋白治疗,3例用Vp-16 泼尼松 环孢素A化疗。7例治愈,好转10例,8例死亡。结论:HPS病因复杂,病情凶险,病死率高,临床及实验室特点类似于恶性组织细胞病,骨髓组织细胞形态学特点有助于两者的鉴别。早期诊断和正确治疗方案是提高HPS患者生存率的关键。

Diagnosis and treatment of hemophagocytic syndrome in children

OBJECTIVE:To increase the awareness of hemophagocytic syndrome(HPS)in children.METHODS:Twenty-four cases of HPS were retrospectively analyzed.RESULTS:HPS in children mostly were caused by infection(15/24,62.5%).The clinical manifestations of HPS included persistent high fever(24/24),hepatosplenomegaly(24/24),decrease of 3 or 2 blood cell lines(24/24),hypertriglyceridemia(12/24),hyperferritinemia(9/10),coagulopathy(18/24),hepatic dysfunction(21/24),benign histiocytic proliferation and phagocytosing hemocytes in bone marrow(24/24).There were therapies of high-dose gamma-globulin(18/24),high-dose methylprednisolone(13/24),and Vp-16(Etoposide) prednisone ciclosporin A(3/24).Seven cases fully recovered(7/24),ten cases improved(10/24),eight cases died(8/24).CONCLUSIONS:Children patients with HPS often undergo an aggressive clinical course resulting in a poor prognosis.The clinical and laboratory features of HPS are similar to malignant histocytosis.Bone marrow histiocytic morphology examination can conduce to identify HPS and malignant histocytosis.Early diagnosis and promptly treatment are critical to improve survival.