Clinical features, genetics, and outcome of pediatric patients with hemophagocytic lymphohistiocytosis in <scp>K</scp>orea: report of a nationwide survey from <scp>K</scp>orea <scp>H</scp>istiocytosis <scp>W</scp>orking <scp>P</scp>arty

Koh, Kyung‐Nam; Im, Ho Joon; Chung, Nack‐Gyun; Cho, Bin; Kang, Hyoung Jin; Shin, Hee Young; Lyu, Chuhl Joo; Yoo, Keon Hee; Koo, Hong Hoe; Kim, Hee‐Jin; Baek, Hee Jo; Kook, Hoon; Yoon, Hoi Soo; Lim, Young Tak; Kim, Heung Sik; Ryu, Kyung Ha; Seo, Jong Jin

doi:10.1111/ejh.12399

Cited by 67 publications

(62 citation statements)

References 27 publications

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“…Our study confirmed that HLH is a disorder that mostly affects young children; however, the age of disease onset in this cohort and other Eastern countries is higher than that reported by Western countries . Although primary HLH is predominant in young children, patients younger than 1 year account for only 17% in our cohort, whereas it was 56% in the study reported by the Histiocyte Society .…”

Section: Discussionsupporting

confidence: 85%

“…EBV infection is reported to be a predominant cause of HLH in Asian populations, and accounts for approximately 40% of patients with HLH in Japan and Korea . In our cohort, approximately 75% of patients had a concomitant EBV infection.…”

Section: Discussionmentioning

confidence: 61%

“…Several challenges remain when the clinicians attempt to make a diagnosis of HLH and to treat those patients. For example, soluble CD25 measurement, natural killer (NK) cell function tests, and genetic sequencing may not be available in time or these tests are even unavailable in many resource‐limited areas . On the other hand, the treatment strategies for HLH vary considerably according to the severity of the disease.…”

Section: Introductionmentioning

confidence: 99%

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Clinical presentation and outcome of pediatric patients with hemophagocytic lymphohistiocytosis in China: A retrospective multicenter study

Wang

et al. 2016

Pediatr Blood Cancer

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Section: Discussionsupporting

confidence: 85%

Section: Discussionmentioning

confidence: 61%

Section: Introductionmentioning

confidence: 99%

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Clinical presentation and outcome of pediatric patients with hemophagocytic lymphohistiocytosis in China: A retrospective multicenter study

Wang

et al. 2016

Pediatr Blood Cancer

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“…7 Renal involvement, particularly AKI, is seen in up to 50% of patients with HLH. [8][9][10] Clinical manifestations include oliguria, azotemia, and nephrotic syndrome. 9 The spectrum of AKI is similar in patients with secondary HLH.…”

Section: Discussionmentioning

confidence: 99%

Direct Reversible Kidney Injury in Familial Hemophagocytic Lymphohistiocytosis Type 3

Malaga-Dieguez¹,

Wu²,

Trachtman³

2015

Journal of the American Society of Nephrology

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Hemophagocytic lymphohistiocytosis is a hyperinflammatory disorder resulting from primary or secondary immune dysfunction. AKI is frequent in severe hemophagocytic lymphohistiocytosis and has been attributed to multiorgan failure or the use of nephrotoxic drugs, but AKI is rarely considered a direct consequence of the disease process. We describe a child with familial hemophagocytic lymphohistiocytosis type 3 who developed AKI requiring prolonged renal replacement therapy because of severe renal inflammation. There was massive infiltration of the renal parenchyma by activated macrophages and cytotoxic T cells, and acute tubular injury. The patient responded to high-dose intravenous methylprednisolone, which resulted in improvement of renal function and discontinuation of renal replacement therapy. This case confirms the occurrence of reversible AKI due to hemophagocytic lymphohistiocytosis-induced activated macrophage infiltration of the renal parenchyma and inflammation.

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“…It accounts for about 40% of all cases of HLH and is the most common type of secondary HLH [13,14]. The diagnosis is es- EBV-HLH in children without underlying disease can be controlled effectively in more than 90% of patients.…”

Section: Acute Ebv-associated Hlhmentioning

confidence: 99%

Epstein-Barr virus-positive T/NK-cell lymphoproliferative diseases in children and adolescents

2018

Precis Future Med

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In primary Epstein-Barr virus (EBV) infection, the virus may infect T-cells or natural killer (NK)-cells and can cause T-and NK-cell (T/NK-cell) lymphoproliferative disease, which encompasses several disease entities with a broad clinicopathological spectrum. T/NKcell lymphoproliferative disease arising during primary EBV infection includes clonal EBV-infected T-cell proliferation in the setting of infectious mononucleosis, EBV-associated hemophagocytic lymphohistiocytosis, and systemic T-cell lymphoma of childhood. Chronic active EBV (CAEBV) infection of the T/NK-cell type is not a simple viral infection but instead is classified as a lymphoproliferative disease. The prototype of CAEBV is a systemic form that exhibits varying degrees of clinical severity depending on the host immunity and EBV factor. Hydroa vacciniforme-like lymphoproliferative disease and mosquito-bite allergy are peculiar cutaneous forms of CAEBV that involve T/ NK-cells, respectively. Abnormal activation and replication of EBV together with the proliferation and clonal expansion of infected cells, depending on the patient's immunological response, play a key role in the pathogenesis of CAEBV of the T/NK-cells type.

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Clinical features, genetics, and outcome of pediatric patients with hemophagocytic lymphohistiocytosis in Korea: report of a nationwide survey from Korea Histiocytosis Working Party

Cited by 67 publications

References 27 publications

Clinical presentation and outcome of pediatric patients with hemophagocytic lymphohistiocytosis in China: A retrospective multicenter study

Clinical presentation and outcome of pediatric patients with hemophagocytic lymphohistiocytosis in China: A retrospective multicenter study

Direct Reversible Kidney Injury in Familial Hemophagocytic Lymphohistiocytosis Type 3

Epstein-Barr virus-positive T/NK-cell lymphoproliferative diseases in children and adolescents

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