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Defective B cell activation

MedGen UID:
375916
•Concept ID:
C1846551
•
Finding
HPO: HP:0005384

Definition

A reduced ability of a B cell to become activated, i.e., the change in morphology and behavior of [from HPO]

Conditions with this feature

Autoimmune lymphoproliferative syndrome type 2B
MedGen UID:
339548
•Concept ID:
C1846545
•
Disease or Syndrome
Caspase 8 deficiency is a syndrome of lymphadenopathy and splenomegaly, marginal elevation of 'double-negative T cells' (DNT; T-cell receptor alpha/beta+, CD4-/CD8-), defective FAS-induced apoptosis, and defective T-, B-, and natural killer (NK)-cell activation, with recurrent bacterial and viral infections (summary by Madkaikar et al., 2011).
See: Condition Record
Autoimmune lymphoproliferative syndrome type 2B

Professional guidelines

PubMed

Recent advances in CAR T-cell toxicity: Mechanisms, manifestations and management.
Brudno JN, Kochenderfer JN
Blood Rev 2019 Mar;34:45-55. Epub 2018 Nov 14 doi: 10.1016/j.blre.2018.11.002. PMID: 30528964Free PMC Article
Unmet Needs in the Pathogenesis and Treatment of Systemic Lupus Erythematosus.
Bakshi J, Segura BT, Wincup C, Rahman A
Clin Rev Allergy Immunol 2018 Dec;55(3):352-367. doi: 10.1007/s12016-017-8640-5. PMID: 28853005Free PMC Article
Primary B cell immunodeficiencies: comparisons and contrasts.
Conley ME, Dobbs AK, Farmer DM, Kilic S, Paris K, Grigoriadou S, Coustan-Smith E, Howard V, Campana D
Annu Rev Immunol 2009;27:199-227. doi: 10.1146/annurev.immunol.021908.132649. PMID: 19302039

Recent clinical studies

Etiology

Evaluation of CARMA1/CARD11 and Bob1 as candidate genes in common variable immunodeficiency.
Tampella G, Baronio M, Vitali M, Soresina A, Badolato R, Giliani S, Plebani A, Lougaris V
J Investig Allergol Clin Immunol 2011;21(5):348-53. PMID: 21905497
Potential advantages and risks of nucleic acid vaccines for infant immunization.
Siegrist CA
Vaccine 1997 Jun;15(8):798-800. doi: 10.1016/s0264-410x(96)00253-8. PMID: 9234517

Therapy

Potential advantages and risks of nucleic acid vaccines for infant immunization.
Siegrist CA
Vaccine 1997 Jun;15(8):798-800. doi: 10.1016/s0264-410x(96)00253-8. PMID: 9234517

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