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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).

Professional guidelines

PubMed

Chang C, Tanaka A, Bowlus C, Gershwin ME
Expert Opin Investig Drugs 2020 Apr;29(4):385-398. Epub 2020 Feb 26 doi: 10.1080/13543784.2020.1733527. PMID: 32102572
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
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

Recent clinical studies

Etiology

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
Siegrist CA
Vaccine 1997 Jun;15(8):798-800. doi: 10.1016/s0264-410x(96)00253-8. PMID: 9234517

Therapy

Siegrist CA
Vaccine 1997 Jun;15(8):798-800. doi: 10.1016/s0264-410x(96)00253-8. PMID: 9234517

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