A Case of Hemophagocytic Lymphohistiocytosis following Refractory Kawasaki Disease.

Research paper by Ye Y Li, Yulong Y Wang, Shen S Li, Mingjing M Liu, Dingding D Wang, Chaoyue C Xu, Luan L Zhang

Indexed on: 27 Feb '20Published on: 26 Feb '20Published in: Klinische Padiatrie


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. © Georg Thieme Verlag KG Stuttgart · New York.