AUTHOR=Wang Leyuan , Yuan Lin , Du Xizi , Zhou Kai , Yang Yu , Qin Qingwu , Yang Liangchun , Xiang Yang , Qu Xiangping , Liu Huijun , Qin Xiaoqun , Liu Chi 

TITLE=A Risk Model Composed of Complete Blood Count, BRAF V600E and MAP2K1 Predicts Inferior Prognosis of Langerhans Cell Histiocytosis in Children

JOURNAL=Frontiers in Oncology

VOLUME=Volume 12 - 2022

YEAR=2022

URL=https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2022.800786

DOI=10.3389/fonc.2022.800786

ISSN=2234-943X

ABSTRACT=Background: In children, langerhans cell histiocytosis (LCH), which is the most prevalent histiocytic disorder, exhibits a wide variety of manifestations as well as outcomes. There is no standard prognosis evaluation system for LCH. We investigated the combined predictive significance of complete blood counts (CBCs), BRAF V600E as well as MAP2K1 in childhood LCH.
Methods: A cohort of 71 childhood LCH patients was retrospectively studied. The prognosis predictive significance of platelet-to-lymphocyte ratio (PLR), neutrophil-to-lymphocyte ratio (NLR), systemic inflammation response index (SIRI), systemic immune inflammation index (SII), BRAF V600E, and MAP2K1 were analyzed.
Results: Histiocyte Society (HS) classification of LCH patients was correlated with NLR, SIRI and progression free survival (PFS), bone involvement was correlated with SIRI, liver involvement was correlated with NLR, SII, SIRI and PFS, spleen involvement was correlated with SIRI, lung involvement was correlated with NLR and PFS, CNS involvement was correlated with PFS, while BRAF V600E was correlated with PLR, NLR, SIRI, SII, PFS, and OS (p＜0.05). MAP2K1 was correlated with NLR, SIRI, PFS, and OS (p＜0.05). Elevated NLR, PLR SIRI and SII predicted inferior PFS and OS (p <0.05). PLR, NLE, SIRI, SII, BRAF V600E and MAP2K1 were used to establish a risk model for stratifying the LCH patients into 3 different risk groups. Respective median PFS for low-, mediate-, as well as high-risk groups were not reached, 26, and 14 months (p＜0.001), and all median OS were not reached (p＜0.001).
Conclusion: The risk model combined with CBCs, BRAF V600E and MAP2K1 might be a promising prognostic system for LCH in children.