Synapse - Somatic mutations in FAS pathway increase hemophagocytic lymphohistiocytosis risk in patients with T- and/or NK-cell lymphoma

Somatic mutations in FAS pathway increase hemophagocytic lymphohistiocytosis risk in patients with T- and/or NK-cell lymphoma Journal Article

Authors:	Liu, Y.; Sardana, R.; Nemirovsky, D.; Frosina, D.; Jungbluth, A.; Johnson, W. T.; Vardhana, S.; Arcila, M.; Horwitz, S. M.; Derkach, A.; Dogan, A.; Xiao, W.
Article Title:	Somatic mutations in FAS pathway increase hemophagocytic lymphohistiocytosis risk in patients with T- and/or NK-cell lymphoma
Abstract:	Although signi ficant progress has been made in understanding the genetic basis of primary hemophagocytic lymphohistiocytosis (HLH), the pathogenesis of secondary HLH, the more prevalent form, remains unclear. Among the various conditions giving rise to secondary HLH, HLH in patients with lymphoma (HLH-L) accounts for a substantial proportion. In this study, we investigated the role of somatic mutations in the pathogenesis of HLH-L in a cohort of patients with T- and/or natural killer -cell lymphoma. We identi fied a 3 -time higher frequency of mutations in FAS pathway in patients with HLH-L. Patients harboring these mutations had a 5 -time increased HLH-L risk. These mutations were independently associated with inferior outcome. Hence, our study demonstrates the association between somatic mutations in FAS pathway and HLH-L. Further studies are warranted on the mechanistic role of these mutations in HLH-L.
Keywords:	impact; gene-mutations; autoimmune lymphoproliferative syndrome
Journal Title:	Blood Advances
Volume:	8
Issue:	12
ISSN:	2473-9529
Publisher:	American Society of Hematology
Date Published:	2024-06-25
Start Page:	3064
End Page:	3075
Language:	English
ACCESSION:	WOS:001256844600001
DOI:	10.1182/bloodadvances.2023011733
PROVIDER:	wos
PMCID:	PMC11222957
PUBMED:	38593227
Notes:	Article -- MSK Cancer Center Support Grant (P30 CA008748) acknowledged in PDF -- MSK corresponding author is Wenbin Xiao -- Source: Wos