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. 2018 Jul 18:15:111-116.
doi: 10.1016/j.jare.2018.05.007. eCollection 2019 Jan.

Clinical characteristics and effective treatments of scrub typhus-associated hemophagocytic lymphohistiocytosis in children

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Clinical characteristics and effective treatments of scrub typhus-associated hemophagocytic lymphohistiocytosis in children

Yi-Mei Jin et al. J Adv Res. .

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is an uncommon and life-threatening disorder that may rarely complicate the clinical course of Orientia tsutsugamushi disease (scrub typhus). Here, we describe the clinical features, laboratory parameters, management, and outcome of 16 children with scrub typhus-associated HLH. All patients satisfied the HLH-2004 diagnostic criteria. All patients had fever of unknown origin and multisystem damage. Raised hepatic transaminases and abnormalities in routine blood test were observed in all children. Imaging tests showed abnormalities in 10 cases. Six patients were treated with intravenous azithromycin for 5 days, and 10 with intravenous chloramphenicol for 7-10 days because of non-response to 3-day azithromycin treatment. Five patients were treated with intravenous albumin and 3 with intravenous immunoglobulin. Two patients with severe symptoms (shortness of breath, cyanosis) were treated with dexamethasone (0.3 mg/kg/d). Fifteen patients recovered completely after 8-22 days of treatment. One patient died. The occurrence of severe complications draws attention to the need for early diagnosis and effective treatment. Anti-rickettsial antibiotic treatment (azithromycin or chloramphenicol) without the need for chemotherapy may be beneficial in such cases, instead of treatment according to the 2004 HLH protocol.

Keywords: Children; Hemophagocytic lymphohistiocytosis; Rickettsial disease.

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Figures

None
Graphical abstract
Fig. 1
Fig. 1
Seasonal variation of scrub typhus with HLH.
Fig. 2
Fig. 2
A: A 17-month-old boy with a 0.3 × 0.3 cm eschar in the left chest. B: A 3-year-old girl with a 0.5 × 0.5 cm ulcer in the right fossa cubitalia. C, D: Hemophagocytes in the bone marrow after bone marrow aspiration. E: Chest X-ray showed discrete, scattered, patchy shadows on both lungs in a 3-year-old girl. F: CT showed bilateral punctuated change and mild pleural effusion in a 2-year-old girl.
Fig. 3
Fig. 3
Complications of patients with scrub typhus-associated HLH.

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