2020
Immunological and Clinical Phenotyping in Primary Antibody Deficiencies: a Growing Disease Spectrum
Shin JJ, Liauw D, Siddiqui S, Lee J, Chung EJ, Steele R, Hsu FI, Price C, Kang I. Immunological and Clinical Phenotyping in Primary Antibody Deficiencies: a Growing Disease Spectrum. Journal Of Clinical Immunology 2020, 40: 592-601. PMID: 32239366, PMCID: PMC7260109, DOI: 10.1007/s10875-020-00773-y.Peer-Reviewed Original ResearchConceptsCommon variable immunodeficiencyInterstitial lung diseasePrimary antibody deficiencyConnective tissue diseaseAntibody deficiencyLow NK cell countHematopoietic malignanciesNon-infectious complicationsNon-infectious manifestationsNK cell countsNatural killer cellsPneumococcal vaccine responsesElectronic medical recordsResultsAll patientsImmune alterationsVaccine responsesKiller cellsTissue diseaseImmunoglobulin levelsVariable immunodeficiencyLung diseasePAD groupIgG deficiencyImmunological profileMedical records
2018
Patients with common variable immunodeficiency with autoimmune cytopenias exhibit hyperplastic yet inefficient germinal center responses
Romberg N, Le Coz C, Glauzy S, Schickel JN, Trofa M, Nolan BE, Paessler M, Xu M, Lambert MP, Lakhani SA, Khokha MK, Jyonouchi S, Heimall J, Takach P, Maglione PJ, Catanzaro J, Hsu FI, Sullivan KE, Cunningham-Rundles C, Meffre E. Patients with common variable immunodeficiency with autoimmune cytopenias exhibit hyperplastic yet inefficient germinal center responses. Journal Of Allergy And Clinical Immunology 2018, 143: 258-265. PMID: 29935219, PMCID: PMC6400323, DOI: 10.1016/j.jaci.2018.06.012.Peer-Reviewed Original ResearchConceptsCommon variable immunodeficiencyVariable immunodeficiencyB cellsCommensal bacteriaIsotype-switched memory B cellsRegulatory T cell frequencyFollicular helper T cellsGC responseIsotype-switched antibodiesT cell frequenciesSubset of patientsT cell compartmentHelper T cellsPeripheral blood samplesMemory B cellsGerminal center responseB cell clonesAutoimmune cytopeniasGC hyperplasiaSerum endotoxemiaExcisional lymphAntibody responseT cellsMucosal microbiotaSomatic hypermutation frequencies