Guo Hai-Yan, Sun Jingmin, Su Yue, Zhang Qin, Hua Ran, Xia Xun, Fang Deng
Department of Pediatrics, The First Affiliated Hospital of Anhui Medical University, Hefei, Anhui 230022, P.R. China.
Centre for Experimental Medicine, School of Medicine, Dentistry and Biomedical Sciences, Queen's University Belfast, Belfast BT12 6BA, Northern Ireland, UK.
Exp Ther Med. 2021 Apr;21(4):396. doi: 10.3892/etm.2021.9827. Epub 2021 Feb 24.
Hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal clinical syndrome frequently complicated by acute kidney injury (AKI) and acute tubular necrosis. Renal thrombotic microangiopathy (TMA) is a specific pathological feature of childhood HLH and few cases have been reported among infants. The present study presents a rare case of HLH with TMA in an infant. A 15-month-old infant with a week-long history of fever was admitted to hospital. The infant presented with AKI and subsequently a reduction in platelet and hemoglobin levels. TMA was diagnosed by kidney biopsy and the clinical, laboratory and bone marrow biopsy findings met the criteria of HLH. Due to a progressive increase in serum creatinine levels, hemodialysis was initiated on the second day following admission. Dexamethasone was administered to treat both the fever and HLH. The patient's body temperature returned to a normal range and platelet and hemoglobin levels were stable after 14 days of admission. Renal function stabilized on day 21. The results of genetic testing did not identify any disease-related variations. Childhood HLH is a severe condition and mortality can be reduced by early diagnosis and correct treatment. For patients with HLH and AKI, the possible role of TMA should be considered. Renal biopsy can help to identify the cause of AKI and can be performed when the patient's condition is stable.
噬血细胞性淋巴组织细胞增生症(HLH)是一种罕见但致命的临床综合征,常并发急性肾损伤(AKI)和急性肾小管坏死。肾血栓性微血管病(TMA)是儿童HLH的一种特殊病理特征,婴儿病例报道较少。本研究报告了1例罕见的婴儿HLH合并TMA病例。一名15个月大、发热一周的婴儿入院。该婴儿出现急性肾损伤,随后血小板和血红蛋白水平下降。通过肾活检诊断为TMA,临床、实验室和骨髓活检结果符合HLH标准。由于血清肌酐水平持续升高,入院第二天开始进行血液透析。给予地塞米松治疗发热和HLH。入院14天后,患者体温恢复正常范围,血小板和血红蛋白水平稳定。第21天时肾功能稳定。基因检测结果未发现任何与疾病相关的变异。儿童HLH病情严重,早期诊断和正确治疗可降低死亡率。对于HLH和AKI患者,应考虑TMA的可能作用。肾活检有助于确定急性肾损伤的病因,可在患者病情稳定时进行。