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

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

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 polyclonal expansion of B cells at an early age with splenomegaly, ly...

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Bibliographic Details
Main Authors: Maya Gupta, Jahnavi Aluri, Mukesh Desai, Madhukar Lokeshwar, Prasad Taur, Michael Lenardo, Jenna Bergerson, Aparna Dalvi, Snehal Mhatre, Manasi Kulkarni, Priyanka Kambli, Manisha Madkaikar
Format: Article
Language:English
Published: Frontiers Media S.A. 2018-06-01
Series:Frontiers in Immunology
Subjects:
CARD11
polyclonal B cell lymphocytosis
NF-κB
GOF mutation
autoimmune lymphoproliferative syndrome
Online Access:https://www.frontiersin.org/article/10.3389/fimmu.2018.01049/full

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