| 王亚秋 丁伟 薛爱国 李伟 宋金莲.37 例儿童噬血细胞综合征的临床分析[J].现代生物医学进展英文版,2014,14(29):5743-5746. |
| 37 例儿童噬血细胞综合征的临床分析 |
| Clinical Analysis of 37 Cases of Hemophagocytic Syndrome in Children |
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| DOI： |
| 中文关键词: 噬血细胞综合征 儿童 临床特点 预后 |
| 英文关键词: Hemophagocytic syndrome Children Clinical characteristics Prognosis |
| 基金项目: |
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| 中文摘要: |
| 目的：分析儿童噬血细胞综合征(hemophagocytic syndrome，HPS)的病因、临床表现、实验室检查结果、治疗和预后特点。方
法：回顾性分析我院收治的37 例HPS 患儿的临床资料。结果：37 例HPS患儿(男24 例、女13 例)，年龄2 月～9岁，5 例(13.5%)有
明显家族史，获得性HPS32 例(86.5%)，包括EB病毒感染16 例、巨细胞病毒感染7 例，其他原因9 例；所有患儿均表现为发热，肝
脾肿大，外周血白细胞、血红蛋白、血小板、白蛋白、纤维蛋白原减低，TG、ALT、AST、LDH、铁蛋白升高；5 例遗传性HPS 患儿死亡
4 例，放弃治疗1 例，剩余32 例患儿中好转20 例(62.5%)，包括痊愈17 例，完全缓解后继续治疗中3 例，未好转12 例(37.5%)，其
中死亡7例，病情危重放弃治疗3 例，复发2 例。12 例未好转病例中，9 例为EBV感染，1 例为肾母细胞瘤，1 例为幼年类风湿性
关节炎合并CMV 感染，1 例原因不明。遗传性HPS的好转率较继发性HPS 明显降低，差异有统计学意义(X2 =5.30，P<0.05)，继发
性HPS中EBV感染者的好转率较非EBV感染者低，差异有统计学意义(X2 =4.80，P<0.05)。结论：及时诊断儿童HPS 并明确其病
因，对该病的治疗及预后具有重要意义。 |
| 英文摘要: |
| Objective:To analyze the pathogen, clinical manifestation, laboratory results, treatment and prognosis of
hemophagocytic syndrome (HPS) in children.Methods:The clinical data of 37 cases of hemophagocytic syndrome in children were
retrospectively analyzed.Results:HPS was diagnosed in 37 children (24 boys and 13 girls), aged from 2 months to 9 years. 5 cases
(13.5%) were familial hemophagocytic lymphohistiocytosis (FLH), while 32 children with HPS were secondary (16 cases were infected
with Epstein Bart virus, 7 cases with cytomegalovirus and 9 cases with other etiopathogenisis). All children had fever and most of the
children had hepatosplenomegaly, leucocytopenia, low haemoglobin, thrombocytopenia, low albumen, hypofibrinogenemia,
hypertriglyceridemia, high ALT, high AST, high LDH and high SF. 4 of the 5 children with FLH died, and 1 gave up treatment. 20
children were improved(17 cases were cured and 3 were undergoing treatment), 10 cases succumbed (7 could not be administrated after
treatment and 3 gave up) and 2 cases relapsed. 12 children (9 affected with EBV) had no improvement. The improvement rate in familial
group was significantly lower than that of the acquired group (X2=5.30, P<0.05). And the improvement rate in EBV infection group was
lower than that in non-EBV infection group in the children with acquired hemophagocytic syndrome (X2=4.80, P<0.05).Conclusion:Prompt diagnosis of HPS in children and identify the cause may be of great importance to the treatment and prognosis of HPS. |
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