Clinical and genetic features of UNC13D deficiency with hypogammaglobulinemia

BackgroundUNC13D deficiency is the most common form of familial hemophagocytic lymphohistiocytosis (FHL) in Asia. Hypogammaglobulinemia is a rare phenotype observed in both patients with FHL3 and sporadic hemophagocytic lymphohistiocytosis (HLH). Our observations suggest that UNC13D deficiency with...

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Bibliographic Details

Published in:	Frontiers in Immunology
Main Authors:	Linyan Xiong, Qin Zhao, Qian Zhao, Zhiyong Zhang, Yunfei An, Xuemei Tang, Hirokazu Kanegane, Xi Yang, Xiaodong Zhao
Format:	Article
Language:	English
Published:	Frontiers Media S.A. 2025-08-01
Subjects:	hemophagocytic lymphohistiocytosis familial hemophagocytic lymphohistiocytosis UNC13D hypogammaglobulinemia antibody deficiency
Online Access:	https://www.frontiersin.org/articles/10.3389/fimmu.2025.1628507/full