摘要
Abstract
Objective To explore the diagnosis, treatment and prognosis of hemophagocytic syndrome <HPS) in children. Methods A retrospective analysis was done for clinical data of 18 children with HPS. Results All the sick children had highfevertl5 Objective To explore the diagnosis, treatment and prognosis of hemophagocytic syndrome <HPS) in children. Methods A retrospective analysis was done for clinical data of 18 children with HPS. Results All the sick children had highfevertl5 children had pancytopeniat 16 had hepatomegaly/splenomegaly, 16 had liver dysfunction , 15 had coagulation disorders, 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 hemophagocytic lymphohistocytosis (HLH) 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 Infection was the major clinical manifestation of HPS observed in this review. Other laboratory detections should be considered when hemophagocytic histocytes are not seen in bone marrow. The therapeutic effect of HLH 2004 protocol is better than HLH 1994 protocol.关键词
淋巴组织细胞增多症,噬血细胞性/儿童/诊断/治疗Key words
lymphohistocysis, hemophagocytic / child/ diagnosis/ therapy分类
临床医学
