Clinical, Immunological, and Molecular Findings in Four Cases of B Cell Expansion With NF-κB and T Cell Anergy Disease for the First Time From India

Abstract excerpt

B cell Expansion with NF-κB and T cell Anergy (BENTA) is a rare primary immunodeficiency disorder caused by mutations in the CARD11 gene and results in constitutive NF-κB activation in B and T cells. Affected patients present with the polyclonal expansion of B cells at an early age with splenomegaly, lymphadenopathy and mild autoimmunity. Here, we discuss four BENTA cases with unusual clinical manifestations not...

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