Intrinsic Plasma Cell Differentiation Defects in B Cell Expansion with NF-κB and T Cell Anergy Patient B Cells

Abstract excerpt

BENTA (B cell Expansion with NF-κB and T cell Anergy) disease is a novel B cell lymphoproliferative disorder caused by germline, gain-of-function mutations in the lymphocyte scaffolding protein CARD11, which drives constitutive NF-κB signaling. Despite dramatic polyclonal expansion of naïve and immature B cells, BENTA patients also present with signs of primary immunodeficiency, including markedly reduced...

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