Article
Clinical features and treatment outcomes of pediatric Langerhans cell histiocytosis with macrophage activation syndrome-hemophagocytic lymphohistiocytosis.
Orphanet journal of rare diseases - 4 Apr 2022
Wang Dong, Chen Xi-Hua, Wei Ang, Zhou Chun-Ju, Zhang Xue, Ma Hong-Hao, Lian Hong-Yun, Zhang Li, Zhang Qing, Huang Xiao-Tong, Wang Chan-Juan, Yang Ying, Liu Wei, Wang Tian-You, Li Zhi-Gang, Cui Lei, Zhang Rui
Abstract excerpt
BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm. A few LCH patients had Macrophage activation syndrome-hemophagocytic lymphohistiocytosis (MAS-HLH), a life-threatening, hyper-inflammatory syndrome. We retrospectively described the clinical-biological characteristics of a series of 28 pediatric LCH patients with MAS-HLH in a single center. We further analyzed the difference in treatment...
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