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Klin Padiatr 2020; 232(02): 73-76
DOI: 10.1055/a-1110-7303
Letter to the Editor
© Georg Thieme Verlag KG Stuttgart · New York

A Case of Hemophagocytic Lymphohistiocytosis following Refractory Kawasaki Disease

Ein Fall von hämophagozytischer Lymphohistiozytose nach refraktärer Kawasaki-Krankheit
Ye Li
1   Department of Pediatrics, Second Hospital of Shandong University, Jinan, China
,
Yulong Wang
1   Department of Pediatrics, Second Hospital of Shandong University, Jinan, China
,
Shen Li
1   Department of Pediatrics, Second Hospital of Shandong University, Jinan, China
,
Mingjing Liu
1   Department of Pediatrics, Second Hospital of Shandong University, Jinan, China
,
Dingding Wang
1   Department of Pediatrics, Second Hospital of Shandong University, Jinan, China
,
Chaoyue Xu
1   Department of Pediatrics, Second Hospital of Shandong University, Jinan, China
,
Luan Zhang
1   Department of Pediatrics, Second Hospital of Shandong University, Jinan, China
› Author Affiliations
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Publication Date:
25 February 2020 (online)

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Introduction

Hemophagocytic lymphohistiocytosis (HLH) is a systemic inflammatory disorder characterized by uncontrolled histiocytic proliferation, hemophagocytosis, macrophage activation, and up-regulation of inflammatory cytokines (Grom AA., Current opinion in rheumatology 2003; 15: 587–590). HLH is usually divided into two types: primary (familial) HLH and secondary (reactive) HLH. Primary HLH is associated with primary immune deficiencies in which specific gene mutations play an important role, such as perforin defects.