Jin Yingkang, Huang Li, Fan Huifeng, Lu Gen, Xu Yi, Wu Zhiyuan
Department of Respiratory Infection, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, Guangdong 510120, P.R. China.
Pediatric Intensive Care Unit, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, Guangdong 510120, P.R. China.
Exp Ther Med. 2016 Oct;12(4):2729-2734. doi: 10.3892/etm.2016.3668. Epub 2016 Sep 6.
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening immune disorder that may be inherited or secondary to infection, malignancy or rheumatological disease. The aim of the present study was to highlight the clinical features of scrub typhus-associated HLH in children. A retrospective study was performed on 6 pediatric patients with scrub typhus-associated HLH. For each patient, medical records were reviewed and analyzed, and demographic, clinical and laboratory data and outcomes were collected. The duration of fever prior to admission ranged between 4 and 12 days. All patients exhibited persistent or intermittent fever, eschar, hepatosplenomegaly and lymphadenopathy at the time of diagnosis. Five patients experienced acute respiratory distress syndrome and disseminated intravascular coagulation (DIC) during hospitalization. Thrombocytopenia was detected in all patients with cytopenia involving two or three cell types, simultaneously. Coagulopathy with prolonged prothrombin time and/or activated partial thromboplastin time were noted in all patients. Markedly elevated serum ferritin levels (>1,500 µg/ml) were identified in all patients. Elevated lactate dehydrogenase (>1,000 U/l) was detected in 4 (66.7%) patients and elevated alanine aminotransferase was exhibited by 5 (83/3%) patients. Lung infiltrates and consolidation were the most common imaging findings. Only 1 patient succumbed, with DIC and multi-organ failure. Of the survivors, 1 patient was lost to follow-up, and the remaining patients are in remission with excellent general health, to date. In conclusion, HLH should be considered in severe pediatric cases of scrub typhus. Upon the early recognition of this syndrome, prompt and supportive treatment in the pediatric intensive care unit are vital.
噬血细胞性淋巴组织细胞增生症（HLH）是一种危及生命的免疫紊乱疾病，可遗传或继发于感染、恶性肿瘤或风湿性疾病。本研究的目的是突出儿童恙虫病相关HLH的临床特征。对6例恙虫病相关HLH的儿科患者进行了回顾性研究。对每位患者的病历进行了回顾和分析，并收集了人口统计学、临床和实验室数据及结果。入院前发热持续时间为4至12天。所有患者在诊断时均表现为持续性或间歇性发热、焦痂、肝脾肿大和淋巴结病。5例患者在住院期间出现急性呼吸窘迫综合征和弥散性血管内凝血（DIC）。所有血细胞减少的患者均检测到血小板减少，同时涉及两到三种细胞类型。所有患者均出现凝血酶原时间和/或活化部分凝血活酶时间延长的凝血病。所有患者血清铁蛋白水平均显著升高（>1500μg/ml）。4例（66.7%）患者检测到乳酸脱氢酶升高（>1000U/l），5例（83.3%）患者出现丙氨酸转氨酶升高。肺部浸润和实变是最常见的影像学表现。仅1例患者死于DIC和多器官功能衰竭。在幸存者中，1例患者失访，其余患者至今病情缓解，总体健康状况良好。总之，严重的儿童恙虫病病例应考虑HLH。一旦早期识别该综合征，在儿科重症监护病房进行及时和支持性治疗至关重要。
