Abstract
BackgroundIn children, Langerhans cell histiocytosis (LCH), which is the most prevalent histiocytic disorder, exhibits a wide variety of manifestations and outcomes. There is no standard prognosis evaluation system for LCH. We investigated the combined predictive significance of complete blood counts (CBCs), BRAF V600E and MAP2K1 in childhood LCH.MethodsA 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.ResultsHistiocyte 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-, and high-risk groups were not reached, 26, and 14 months (p <0.001), and all median OS were not reached (p <0.001).ConclusionThe risk model combined with CBCs, BRAF V600E, and MAP2K1 might be a promising prognostic system for LCH in children.
Highlights
Langerhans cell histiocytosis (LCH), referred to as histiocytosis, defines a collection of histiocytosis disorders of unknown cause
Histiocyte Society (HS) classification of LCH patients was correlated with neutrophil-tolymphocyte ratio (NLR), systemic inflammation response index (SIRI), and progression free survival (PFS), bone involvement was correlated with SIRI, liver involvement was correlated with NLR, systemic immune inflammation index (SII), SIRI, and PFS, spleen involvement was correlated with SIRI, lung involvement was correlated with NLR and PFS, central nervous system (CNS) involvement was correlated with PFS, while BRAF V600E was correlated with platelet-to-lymphocyte ratio (PLR), NLR, SIRI, SII, PFS, and Overall survival (OS) (p
The risk model combined with complete blood counts (CBCs), BRAF V600E, and MAP2K1 might be a promising prognostic system for LCH in children
Summary
Langerhans cell histiocytosis (LCH), referred to as histiocytosis, defines a collection of histiocytosis disorders of unknown cause. LCH exhibits various manifestations, from single indolent lesions to explosive multisystem diseases [1]. The prognoses of LCH patients have slightly improved in the past decades, the standard treatment regimen fails to cure more than 50% patients with the multisystem disease [2]. There is no widely accepted prognosis evaluation system for LCH. Langerhans cell histiocytosis (LCH), which is the most prevalent histiocytic disorder, exhibits a wide variety of manifestations and outcomes. There is no standard prognosis evaluation system for LCH. We investigated the combined predictive significance of complete blood counts (CBCs), BRAF V600E and MAP2K1 in childhood LCH
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