儿童恙虫病继发噬血细胞综合征的临床特点

Clinical characteristics of hemophagocytic syndrome secondary to tsutsugamushi disease in children

目的探讨儿童恙虫病继发噬血细胞综合征的临床特点。方法回顾分析1例恙虫病继发噬血细胞综合征患儿的临床资料,并检索国内外期刊数据库,收集32例儿童恙虫病继发噬血细胞综合征的临床资料进行分析。结果 9个月女婴,因发热9天伴抽搐1次入院,入院第3天根据其符合HLH-2004诊断标准8条中的5条,即发热、脾肿大、血细胞减少、血清铁蛋白升高、低纤维蛋白原血症或高脂血症,并有特征性焦痂及血恙虫病抗体阳性,确诊为恙虫病继发噬血细胞综合征。确诊后予阿奇霉素抗感染治疗,3天后体温降至正常,复查各项指标逐渐改善。文献检索到32例(不含本文1例)恙虫病继发噬血细胞综合征患儿,均为亚洲人,男、女各16例,年龄为2个月～11岁;24例患儿有焦痂,30例发现脾大,30例血白蛋白降低,几乎所有患儿都有血细胞减少和血清铁蛋白水平升高。31例患儿病初被误诊。27例采用适当的抗生素治疗,16例联合使用丙种球蛋白,21例使用激素,1例进行了依托泊苷化疗。多数患儿治疗24小时至4天未再发热,5例死亡。结论恙虫病继发噬血细胞综合征常常伴有血清白蛋白降低,需要适当的抗生素及免疫调节治疗,多数预后良好。

Objective To explore the clinical features of haemophagocytic syndrome secondary to tsutsugamushi disease in children.Method The clinical data of a child with hemophagocytic syndrome secondary to tsutsugamushi disease were retrospectively analyzed.Domestic and foreign periodical databases were searched,and the clinical data of hemophagocytic syndrome secondary to tsutsugamushi disease in 32 children were collected and analyzed.Results A 9-month-old girl was admitted due to fever of unknown origin for 9 days with an episode of seizure.She was diagnosed of hemophagocytic syndrome secondary to tsutsugamushi disease 3 days after admission because 5 clinical symptoms including fever,splenomegaly,hematocytopenia,serum ferritin elevation,hypofibrinogenemia or hyperlipemia were among the HLH-2004 diagnosis criteria(8 items),together with its characteristic eschar and positive antibodies of scrub typhus.The body temperature dropped to normal 3 days after anti-infection treatment with azithromycin and all the indicators were gradually improved.In the search,a total of 32 patients(excluding this child)with hemophagocytic syndrome secondary to tsutsugamushi disease were retrieved,all of whom were Asians,16 males and 16 females,aged from 2 months to 11 years.Eschar was found in 24 cases,splenomegaly in 30 cases,and decreased serum albumin in 30 cases.Decreased blood cells and elevated serum ferritin levels were observed in almost all of the children.Thirty-one children were misdiagnosed at first.Twenty-seven children were treated with appropriate antibiotics,16 with a combination of gamma globulin,21 with corticosteroids,and only 1 with etoposide chemotherapy.Most children no longer had a fever after treatment from 24 hours to 4 days,and 5 died.Conclusion The hemophagocytic syndrome secondary to tsutsugamushi disease is often accompanied by a decrease in albumin and requires appropriate antibiotic and immunomodulatory treatment,and most of the patients have good prognosis.