Clinical features and treatment outcomes of pediatric Langerhans cell histiocytosis with macrophage activation syndrome-hemophagocytic lymphohistiocytosis

Clinical features and treatment outcomes of pediatric Langerhans cell histiocytosis with macrophage activation syndrome-hemophagocytic lymphohistiocytosis

Abstract 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 characteristic...

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Bibliographic Details
Main Authors: Dong Wang, Xi-Hua Chen, Ang Wei, Chun-Ju Zhou, Xue Zhang, Hong-Hao Ma, Hong-Yun Lian, Li Zhang, Qing Zhang, Xiao-Tong Huang, Chan-Juan Wang, Ying Yang, Wei Liu, Tian-You Wang, Zhi-Gang Li, Lei Cui, Rui Zhang
Format: Article
Language:English
Published: BMC 2022-04-01
Series:Orphanet Journal of Rare Diseases
Subjects:
Langerhans cell histiocytosis
Macrophage activation syndrome-hemophagocytic lymphohistiocytosis
BRAF-V600E mutation
Dabrafenib
Outcome
Online Access:https://doi.org/10.1186/s13023-022-02276-y

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