摘要
目的探讨儿童噬血细胞综合征(HPS)的诊断、治疗及预后。方法对我科诊治18例HPS病儿的临床资料进行回顾性分析。结果所有病儿均有高热,15例伴有血细胞减少,16例伴有肝脾大,16例伴有肝功能异常,15例伴有凝血功能障碍,16例伴有高三酰甘油血症。骨髓检查均可见噬血组织细胞。原发性1例,继发性17例,其中感染相关14例。明确诊断后6例给予噬血细胞性淋巴组织细胞增生症(HLH)1994方案化疗,9例给予HLH 2004方案化疗。治愈或好转15例,死亡3例。HLH 2004方案疗效明显优于1994方案,差异有显著意义(uc=2.011,P<0.05)。结论 HPS临床表现以感染多见,骨髓中未找到噬血细胞者应结合其他实验室检测结果综合考虑。治疗效果以HLH 2004方案较好。
Objective To explore the diagnosis, treatment and prognosis of hemophagocytic syndrome （HPS） in chil dren. Methods A retrospective analysis was done for clinical data of 18 children with HPS. Results All the sick children had high fever, 15 children had pancytopenia, 16 had hepatomegaly/splenomegaly, 16 had liver dysfunction, 15 had coagulation disor- ders, and 16 had hypertriglyceridemia. Hemophagocytic histocytes could be seen in bone marrow. One case was primary,17 were secondary, of which, 14 were infection-related. Upon confirmation of the diagnosis, six patients received chemotherapy of hemoph agocytic lymphohistocytosis （HI.H） 1994 protocol, and nine received HLH 2004 protocol. After treatment, 15 cases were cured or improved, and three died. HLH 2004 protocol was better than HLH 1994 protocol （Uc=2. 011,P〈0.05）. Conclusion Infec- tion was the major clinical manifestation of HPS observed in this review. Other laboratory detections should be considered when he- mophagocytic histocytes are not seen in bone marrow. The therapeutic effect of HLH 2004 protocol is better than HLH 1994 protocol.
出处
《青岛大学医学院学报》
CAS
2012年第3期266-268,共3页
Acta Academiae Medicinae Qingdao Universitatis
