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scrub 伤寒相关噬血细胞性淋巴组织细胞增生症:在儿科年龄组中并非罕见。

Scrub Typhus-Associated Hemophagocytic Lymphohistiocytosis: Not a Rare Entity in Pediatric Age Group.

机构信息

Department of Pediatrics, Medical College and Hospital, Kolkata, West Bengal 700073, India.

出版信息

J Trop Pediatr. 2021 Jan 29;67(1). doi: 10.1093/tropej/fmab001.

DOI:10.1093/tropej/fmab001
PMID:33547467
Abstract

BACKGROUND

Our goal was to study the demographic, clinical and laboratory profile and outcome of scrub typhus-associated hemophagocytic lymphohistiocytosis (HLH) in the pediatric age group.

METHODS

We conducted a prospective observational study in a tertiary care teaching hospital over a period of 1 year. Children in the age group of 1 month to 12 years with IgM ELISA positive for scrub typhus were included in the study. HLH was diagnosed using HLH-2004 criteria. Demographic, clinical and laboratory profile, treatment and outcome of HLH patients were noted and also compared with non-HLH scrub typhus patients.

RESULTS

Among 58 children with scrub typhus infection, 18 had HLH. The mean age of patients with HLH was 35.3 ± 44.8 months and 61% were male. Anemia, thrombocytopenia and hyperferritinemia were seen in all the patients. Hypertriglyceridemia, hypofibrinogenemia and coagulopathy were noted in 78%, 56% and 44%, respectively. All the patients were treated with intravenous doxycycline for an average duration of 9.5 days. Intravenous immunoglobulin and methylprednisolone were given in 33% and 22% cases, respectively. Complications like acute respiratory distress syndrome (ARDS) (p = 0.001) and MODS (p = 0.004) were significantly high in the HLH group. Younger age (<3 years), fever > 7 days, presence of convulsion, ARDS and MODS were the clinical predictors of scrub typhus-associated HLH.

CONCLUSION

HLH in scrub typhus infected children is being increasingly recognized. Younger age, prolonged fever, presence of convulsion, ARDS and MODS should alert clinicians of the risk of HLH. Treating the primary cause usually cures the disease and immunomodulator therapy need not be routinely administered.

摘要

背景

我们的目标是研究小儿年龄组中恙虫病相关噬血细胞性淋巴组织细胞增生症(HLH)的人口统计学、临床和实验室特征及转归。

方法

我们在一家三级教学医院进行了为期 1 年的前瞻性观察性研究。纳入年龄在 1 个月至 12 岁之间、酶联免疫吸附试验 IgM 阳性的恙虫病患儿。采用 HLH-2004 标准诊断 HLH。记录 HLH 患者的人口统计学、临床和实验室特征、治疗及转归,并与非 HLH 恙虫病患者进行比较。

结果

在 58 例恙虫病感染患儿中,有 18 例发生 HLH。HLH 患者的平均年龄为 35.3±44.8 个月,61%为男性。所有患者均存在贫血、血小板减少和高铁蛋白血症。78%、56%和 44%的患者分别存在高甘油三酯血症、低纤维蛋白原血症和凝血功能障碍。所有患者均接受静脉注射多西环素治疗,平均疗程为 9.5 天。33%和 22%的患者分别接受了静脉注射免疫球蛋白和甲基强的松龙治疗。HLH 组的急性呼吸窘迫综合征(ARDS)(p=0.001)和多器官功能障碍综合征(MODS)(p=0.004)并发症明显较高。年龄较小(<3 岁)、发热>7 天、存在惊厥、ARDS 和 MODS 是恙虫病相关 HLH 的临床预测因素。

结论

在感染恙虫病的儿童中,HLH 越来越受到关注。年龄较小、发热时间较长、存在惊厥、ARDS 和 MODS 时,临床医生应警惕 HLH 的发生风险。治疗原发病通常可治愈疾病,且不一定需要常规使用免疫调节剂治疗。

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