Article
Manifestations of B-cell expansion with NF-κB and T-cell anergy disease overlapping with hemophagocytic lymphohistiocytosis.
Scandinavian journal of immunology - 1 Apr 2023
Xiao Haijuan, Mou Wenjun, Zhang Rui, Guo Xin, Chen Heying, Zhang Liqiang, Liu Gang, Gui Jingang
Abstract excerpt
B cell expansion with NF-κB and T cell anergy (BENTA) is a disease genetically linked with heterozygous gain-of-function (GOF) mutations in the CARD11 gene with an autosomal dominant expression. Hemophagocytic lymphohistiocytosis (HLH) is a heterogeneous group of disorders characterized by systemic inflammation and hypercytokinaemia. Some BENTA patients share similar clinical manifestations as HLH in various...
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