Thrombotic microangiopathy led to acute kidney injury in an infant with hemophagocytic lymphohistiocytosis: A case report

Guo,Hai-Yan; Sun,Jingmin; Su,Yue; Zhang,Qin; Hua,Ran; Xia,Xun; Fang,Deng

doi:10.3892/etm.2021.9827

Thrombotic microangiopathy led to acute kidney injury in an infant with hemophagocytic lymphohistiocytosis: A case report

Authors:
- Hai-Yan Guo
- Jingmin Sun
- Yue Su
- Qin Zhang
- Ran Hua
- Xun Xia
- Deng Fang
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Affiliations: 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, Department of Nephrology, Anhui Provincial Children's Hospital, Hefei, Anhui 230022, P.R. China
Published online on: February 24, 2021 https://doi.org/10.3892/etm.2021.9827
Article Number: 396
Copyright: © Guo et al. This is an open access article distributed under the terms of Creative Commons Attribution License.

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Abstract

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.

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