Published in N Engl J Med on May 04, 2006
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Dock8 mutations cripple B cell immunological synapses, germinal centers and long-lived antibody production. Nat Immunol (2009) 2.29
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LPS-responsive beige-like anchor (LRBA) gene mutation in a family with inflammatory bowel disease and combined immunodeficiency. J Allergy Clin Immunol (2012) 1.26
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CD19: a promising B cell target for rheumatoid arthritis. Nat Rev Rheumatol (2009) 1.24
Germline mutations in NFKB2 implicate the noncanonical NF-κB pathway in the pathogenesis of common variable immunodeficiency. Am J Hum Genet (2013) 1.23
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Exhaustion of bacteria-specific CD4 T cells and microbial translocation in common variable immunodeficiency disorders. J Exp Med (2014) 1.02
Clinical heterogeneity can hamper the diagnosis of patients with ZAP70 deficiency. Eur J Pediatr (2008) 1.00
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Thymic and bone marrow output in patients with common variable immunodeficiency. J Clin Immunol (2011) 0.99
A c-Myc and surface CD19 signaling amplification loop promotes B cell lymphoma development and progression in mice. J Immunol (2012) 0.99
Autoimmune cytopenias in common variable immunodeficiency. Front Immunol (2012) 0.97
Comparison of diagnostic criteria for common variable immunodeficiency disorder. Front Immunol (2014) 0.95
A machine learning approach for genome-wide prediction of morbid and druggable human genes based on systems-level data. BMC Genomics (2010) 0.95
Unique DNA repair gene variations and potential associations with the primary antibody deficiency syndromes IgAD and CVID. PLoS One (2010) 0.93
The darker side of follicular helper T cells: from autoimmunity to immunodeficiency. Cell Mol Immunol (2012) 0.93
Rationale of anti-CD19 immunotherapy: an option to target autoreactive plasma cells in autoimmunity. Arthritis Res Ther (2012) 0.91
Novel mutations in TACI (TNFRSF13B) causing common variable immunodeficiency. J Clin Immunol (2009) 0.91
Interferon signature in the blood in inflammatory common variable immune deficiency. PLoS One (2013) 0.90
Genetics of hypogammaglobulinemia: what do we really know? Curr Opin Immunol (2009) 0.90
Genetic defects in common variable immunodeficiency. Int J Immunogenet (2007) 0.90
Adult-onset presentations of genetic immunodeficiencies: genes can throw slow curves. Curr Opin Infect Dis (2010) 0.89
TACI mutations and impaired B-cell function in subjects with CVID and healthy heterozygotes. J Allergy Clin Immunol (2012) 0.89
Screening of functional and positional candidate genes in families with common variable immunodeficiency. BMC Immunol (2008) 0.89
Simultaneous downregulation of KLF5 and Fli1 is a key feature underlying systemic sclerosis. Nat Commun (2014) 0.88
Association of CLEC16A with human common variable immunodeficiency disorder and role in murine B cells. Nat Commun (2015) 0.88
Human B cell defects in perspective. Immunol Res (2012) 0.86
Application of whole genome and RNA sequencing to investigate the genomic landscape of common variable immunodeficiency disorders. Clin Immunol (2015) 0.86
TACI mutations and disease susceptibility in patients with common variable immunodeficiency. Clin Exp Immunol (2008) 0.86
Common Variable Immunodeficiency and Circulating TFH. J Immunol Res (2016) 0.85
CD19 and CD32b differentially regulate human B cell responsiveness. J Immunol (2014) 0.85
The Principles of Engineering Immune Cells to Treat Cancer. Cell (2017) 0.85
Exome Sequencing Analysis Reveals Variants in Primary Immunodeficiency Genes in Patients With Very Early Onset Inflammatory Bowel Disease. Gastroenterology (2015) 0.84
Clinical variability of family members with the C104R mutation in transmembrane activator and calcium modulator and cyclophilin ligand interactor (TACI). J Clin Immunol (2012) 0.83
Rare variants at 16p11.2 are associated with common variable immunodeficiency. J Allergy Clin Immunol (2015) 0.82
CD19 controls Toll-like receptor 9 responses in human B cells. J Allergy Clin Immunol (2015) 0.82
CD19 chimeric antigen receptor (CD19 CAR)-redirected adoptive T-cell immunotherapy for the treatment of relapsed or refractory B-cell Non-Hodgkin's Lymphomas. Am J Cancer Res (2016) 0.81
A custom 148 gene-based resequencing chip and the SNP explorer software: new tools to study antibody deficiency. Hum Mutat (2010) 0.81
Blinatumomab for the treatment of acute lymphoblastic leukemia. Invest New Drugs (2015) 0.80
Three different classifications, B lymphocyte subpopulations, TNFRSF13B (TACI), TNFRSF13C (BAFF-R), TNFSF13 (APRIL) gene mutations, CTLA-4 and ICOS gene polymorphisms in Turkish patients with common variable immunodeficiency. J Clin Immunol (2012) 0.80
Critical role of SHP2 (PTPN11) signaling in germinal center-derived lymphoma. Haematologica (2014) 0.80
Immunopathogenesis of granulomas in chronic autoinflammatory diseases. Clin Transl Immunology (2016) 0.80
Common variable immune deficiency in children--clinical characteristics varies depending on defect in peripheral B cell maturation. J Clin Immunol (2013) 0.79
Toll-like receptor function in primary B cell defects. Front Biosci (Elite Ed) (2012) 0.79
Targeting CD19 in B-cell lymphoma: emerging role of SAR3419. Cancer Manag Res (2013) 0.79
The role of genomics in common variable immunodeficiency disorders. Clin Exp Immunol (2017) 0.79
The clinical utility of molecular diagnostic testing for primary immune deficiency disorders: a case based review. Allergy Asthma Clin Immunol (2010) 0.79
Monozygotic twins discordant for common variable immunodeficiency reveal impaired DNA demethylation during naïve-to-memory B-cell transition. Nat Commun (2015) 0.78
Immunological characteristics and two novel mutations in TACI in a cohort of 28 pediatric patients with common variable immunodeficiency. J Clin Immunol (2011) 0.78
A mutation in the human tetraspanin CD81 gene is expressed as a truncated protein but does not enable CD19 maturation and cell surface expression. J Clin Immunol (2015) 0.78
IgH sequences in common variable immune deficiency reveal altered B cell development and selection. Sci Transl Med (2015) 0.77
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Bone marrow transcriptome and epigenome profiles of equine common variable immunodeficiency patients unveil block of B lymphocyte differentiation. Clin Immunol (2015) 0.77
Novel sequencing-based strategies for high-throughput discovery of genetic mutations underlying inherited antibody deficiency disorders. Curr Allergy Asthma Rep (2011) 0.77
Reduced CD19 expression and decreased memory B cell numbers in transient hypogammaglobulinemia of infancy. Clin Exp Med (2012) 0.77
Maintaining intestinal health: the genetics and immunology of very early onset inflammatory bowel disease. Cell Mol Gastroenterol Hepatol (2015) 0.77
Parental consanguinity is associated with a severe phenotype in common variable immunodeficiency. J Clin Immunol (2011) 0.77
Systemic lupus erythematosus as a first presentation of common variable immunodeficiency associated with infrequent mannose-binding lectin gene polymorphisms. Rheumatol Int (2009) 0.76
Induction of interleukin-6 production by rituximab in human B cells. Arthritis Rheumatol (2014) 0.76
CD19 as a molecular target in CNS autoimmunity. Acta Neuropathol (2014) 0.76
Gastrointestinal Pathologic Abnormalities in Pediatric- and Adult-Onset Common Variable Immunodeficiency. Dig Dis Sci (2015) 0.76
Common variable immunodeficiency associated with microdeletion of chromosome 1q42.1-q42.3 and inositol 1,4,5-trisphosphate kinase B (ITPKB) deficiency. Clin Transl Immunology (2016) 0.76
B cell responses to CpG correlate with CXCL16 expression levels in common variable immunodeficiency. ScientificWorldJournal (2012) 0.76
Disabled receptor signaling and new primary immunodeficiency disorders. N Engl J Med (2006) 0.76
Rac-mediated Stimulation of Phospholipase Cγ2 Amplifies B Cell Receptor-induced Calcium Signaling. J Biol Chem (2015) 0.76
Antigen-based immunotherapy for the treatment of acute lymphoblastic leukemia: the emerging role of blinatumomab. Immunotargets Ther (2014) 0.75
Proteomic analysis of sera from common variable immunodeficiency patients undergoing replacement intravenous immunoglobulin therapy. J Biomed Biotechnol (2011) 0.75
Expression of activation-induced cytidine deaminase gene in B lymphocytes of patients with common variable immunodeficiency. Iran J Pediatr (2013) 0.75
STAT3 mutations in the hyper-IgE syndrome. N Engl J Med (2007) 8.99
Inflammatory bowel disease and mutations affecting the interleukin-10 receptor. N Engl J Med (2009) 7.53
A homozygous CARD9 mutation in a family with susceptibility to fungal infections. N Engl J Med (2009) 5.20
Deficiency of Th17 cells in hyper IgE syndrome due to mutations in STAT3. J Exp Med (2008) 4.76
Poor-prognosis colon cancer is defined by a molecularly distinct subtype and develops from serrated precursor lesions. Nat Med (2013) 4.22
The 3D structure of the immunoglobulin heavy-chain locus: implications for long-range genomic interactions. Cell (2008) 3.92
Common variable immunodeficiency disorders: division into distinct clinical phenotypes. Blood (2008) 3.44
Increased colorectal cancer risk during follow-up in patients with hyperplastic polyposis syndrome: a multicentre cohort study. Gut (2009) 3.34
Loss of interleukin-10 signaling and infantile inflammatory bowel disease: implications for diagnosis and therapy. Gastroenterology (2012) 3.28
Large deletions and point mutations involving the dedicator of cytokinesis 8 (DOCK8) in the autosomal-recessive form of hyper-IgE syndrome. J Allergy Clin Immunol (2009) 3.13
HAX1 deficiency causes autosomal recessive severe congenital neutropenia (Kostmann disease). Nat Genet (2006) 3.03
ICOS deficiency is associated with a severe reduction of CXCR5+CD4 germinal center Th cells. J Immunol (2006) 2.99
The human syndrome of dendritic cell, monocyte, B and NK lymphoid deficiency. J Exp Med (2011) 2.96
A novel human primary immunodeficiency syndrome caused by deficiency of the endosomal adaptor protein p14. Nat Med (2006) 2.85
Role of truncating mutations in MME gene in fetomaternal alloimmunisation and antenatal glomerulopathies. Lancet (2004) 2.82
The transmembrane activator TACI triggers immunoglobulin class switching by activating B cells through the adaptor MyD88. Nat Immunol (2010) 2.55
New insights on human T cell development by quantitative T cell receptor gene rearrangement studies and gene expression profiling. J Exp Med (2005) 2.44
Hyperplastic polyps and sessile serrated adenomas as a phenotypic expression of MYH-associated polyposis. Gastroenterology (2008) 2.41
Wnt3a deficiency irreversibly impairs hematopoietic stem cell self-renewal and leads to defects in progenitor cell differentiation. Blood (2008) 2.37
A syndrome with congenital neutropenia and mutations in G6PC3. N Engl J Med (2009) 2.30
Late MRD response determines relapse risk overall and in subsets of childhood T-cell ALL: results of the AIEOP-BFM-ALL 2000 study. Blood (2011) 2.18
Canonical wnt signaling regulates hematopoiesis in a dosage-dependent fashion. Cell Stem Cell (2011) 2.18
CD20 deficiency in humans results in impaired T cell-independent antibody responses. J Clin Invest (2009) 2.15
Human memory B cells originate from three distinct germinal center-dependent and -independent maturation pathways. Blood (2011) 2.15
Infant colitis--it's in the genes. Lancet (2010) 2.10
Final report of the efficacy and safety of gemtuzumab ozogamicin (Mylotarg) in patients with CD33-positive acute myeloid leukemia in first recurrence. Cancer (2005) 2.08
Soluble BAFF levels inversely correlate with peripheral B cell numbers and the expression of BAFF receptors. J Immunol (2011) 2.08
Replication history of B lymphocytes reveals homeostatic proliferation and extensive antigen-induced B cell expansion. J Exp Med (2007) 2.05
Protease-activated receptor 2 suppresses lymphangiogenesis and subsequent lymph node metastasis in a murine pancreatic cancer model. J Pathol (2014) 2.02
Autosomal recessive hyperimmunoglobulin E syndrome: a distinct disease entity. J Pediatr (2004) 2.01
B-cell activating factor receptor deficiency is associated with an adult-onset antibody deficiency syndrome in humans. Proc Natl Acad Sci U S A (2009) 1.95
Deleterious mutations in LRBA are associated with a syndrome of immune deficiency and autoimmunity. Am J Hum Genet (2012) 1.94
Circulating CD21low B cells in common variable immunodeficiency resemble tissue homing, innate-like B cells. Proc Natl Acad Sci U S A (2009) 1.91
Reexamining the role of TACI coding variants in common variable immunodeficiency and selective IgA deficiency. Nat Genet (2007) 1.87
TACItly changing tunes: farewell to a yin and yang of BAFF receptor and TACI in humoral immunity? New genetic defects in common variable immunodeficiency. Curr Opin Allergy Clin Immunol (2005) 1.86
Genetic CD21 deficiency is associated with hypogammaglobulinemia. J Allergy Clin Immunol (2011) 1.86
The phenotype of human STK4 deficiency. Blood (2012) 1.84
A DNA-PKcs mutation in a radiosensitive T-B- SCID patient inhibits Artemis activation and nonhomologous end-joining. J Clin Invest (2008) 1.82
Analyzing primary Hodgkin and Reed-Sternberg cells to capture the molecular and cellular pathogenesis of classical Hodgkin lymphoma. Blood (2012) 1.82
CD81 gene defect in humans disrupts CD19 complex formation and leads to antibody deficiency. J Clin Invest (2010) 1.79
Origin and pathogenesis of nodular lymphocyte-predominant Hodgkin lymphoma as revealed by global gene expression analysis. J Exp Med (2008) 1.74
Late recurrence of childhood T-cell acute lymphoblastic leukemia frequently represents a second leukemia rather than a relapse: first evidence for genetic predisposition. J Clin Oncol (2011) 1.71
DOCK8 functions as an adaptor that links TLR-MyD88 signaling to B cell activation. Nat Immunol (2012) 1.70
Human ICOS deficiency abrogates the germinal center reaction and provides a monogenic model for common variable immunodeficiency. Blood (2005) 1.63
Transmembrane activator and calcium-modulating cyclophilin ligand interactor mutations in common variable immunodeficiency: clinical and immunologic outcomes in heterozygotes. J Allergy Clin Immunol (2007) 1.62
The bone morphogenetic protein pathway is inactivated in the majority of sporadic colorectal cancers. Gastroenterology (2008) 1.61
Ig gene rearrangement steps are initiated in early human precursor B cell subsets and correlate with specific transcription factor expression. J Immunol (2005) 1.61
Chemokine/chemokine receptor interactions in extramedullary leukaemia of the skin in childhood AML: differential roles for CCR2, CCR5, CXCR4 and CXCR7. Pediatr Blood Cancer (2010) 1.59
A new type of radiosensitive T-B-NK+ severe combined immunodeficiency caused by a LIG4 mutation. J Clin Invest (2005) 1.59
Management of Epstein-Barr virus (EBV) reactivation after allogeneic stem cell transplantation by simultaneous analysis of EBV DNA load and EBV-specific T cell reconstitution. Clin Infect Dis (2006) 1.59
Idiopathic CD4+ T lymphopenia without autoimmunity or granulomatous disease in the slipstream of RAG mutations. Blood (2011) 1.53
The effect of a novel recombination between the homeobox gene NKX2-5 and the TRD locus in T-cell acute lymphoblastic leukemia on activation of the NKX2-5 gene. Haematologica (2006) 1.52
Anti-TNF treatment blocks the induction of T cell-dependent humoral responses. Ann Rheum Dis (2012) 1.51
Cyclin D1 and CDK4 activity contribute to the undifferentiated phenotype in neuroblastoma. Cancer Res (2008) 1.50
Defects along the T(H)17 differentiation pathway underlie genetically distinct forms of the hyper IgE syndrome. J Allergy Clin Immunol (2009) 1.49
"A rose is a rose is a rose," but CVID is Not CVID common variable immune deficiency (CVID), what do we know in 2011? Adv Immunol (2011) 1.49
Differential Noxa/Mcl-1 balance in peripheral versus lymph node chronic lymphocytic leukemia cells correlates with survival capacity. Blood (2006) 1.47
Comparison of imiquimod, topical fluorouracil, and electrocautery for the treatment of anal intraepithelial neoplasia in HIV-positive men who have sex with men: an open-label, randomised controlled trial. Lancet Oncol (2013) 1.47
Detection of minimal residual disease identifies differences in treatment response between T-ALL and precursor B-ALL. Blood (2002) 1.45
[The ESID Online Database for primary immunodeficiencies. First analyses with regard to Germany and Europe]. Med Klin (Munich) (2008) 1.42
Wnt target genes identified by DNA microarrays in immature CD34+ thymocytes regulate proliferation and cell adhesion. J Immunol (2004) 1.41
Antigen receptors and somatic hypermutation in B-cell chronic lymphocytic leukemia with Richter's transformation. Haematologica (2006) 1.40
Mitochondrial DNA and sperm quality in patients under antiretroviral therapy. AIDS (2003) 1.38
Mutations in STAT3 and diagnostic guidelines for hyper-IgE syndrome. J Allergy Clin Immunol (2010) 1.36
A mutated B cell chronic lymphocytic leukemia subset that recognizes and responds to fungi. J Exp Med (2013) 1.32
Among B cell non-Hodgkin's lymphomas, MALT lymphomas express a unique antibody repertoire with frequent rheumatoid factor reactivity. J Exp Med (2005) 1.32
DOCK8 deficiency: clinical and immunological phenotype and treatment options - a review of 136 patients. J Clin Immunol (2015) 1.32
Multiple infusions of mesenchymal stromal cells induce sustained remission in children with steroid-refractory, grade III-IV acute graft-versus-host disease. Br J Haematol (2013) 1.31
Clinical outcome in IL-10- and IL-10 receptor-deficient patients with or without hematopoietic stem cell transplantation. J Allergy Clin Immunol (2012) 1.30
Novel HAX1 mutations in patients with severe congenital neutropenia reveal isoform-dependent genotype-phenotype associations. Blood (2008) 1.30
Human thymus contains multipotent progenitors with T/B lymphoid, myeloid, and erythroid lineage potential. Blood (2005) 1.29
IL-10 and IL-10 receptor defects in humans. Ann N Y Acad Sci (2011) 1.29
Inherited and acquired immunodeficiencies underlying tuberculosis in childhood. Immunol Rev (2015) 1.29
mTOR inhibitor treatment of pancreatic cancer in a patient With Peutz-Jeghers syndrome. J Clin Oncol (2010) 1.29
Efficacy and safety of hizentra®, a new 20% immunoglobulin preparation for subcutaneous administration, in pediatric patients with primary immunodeficiency. J Clin Immunol (2011) 1.28
Identification of a homozygous deletion in the AP3B1 gene causing Hermansky-Pudlak syndrome, type 2. Blood (2006) 1.28
Unusual cause of respiratory distress: Chilaiditi syndrome. Pediatr Int (2004) 1.26
Wnt signaling in the thymus is regulated by differential expression of intracellular signaling molecules. Proc Natl Acad Sci U S A (2006) 1.24
Mutations in ZBTB24 are associated with immunodeficiency, centromeric instability, and facial anomalies syndrome type 2. Am J Hum Genet (2011) 1.24
An update on the hyper-IgE syndromes. Arthritis Res Ther (2012) 1.23