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https://www.readbyqxmd.com/read/29204088/terminally-differentiated-memory-t-cells-are-increased-in-patients-with-common-variable-immunodeficiency-and-selective-iga-deficiency
#1
Jana Nechvatalova, Tomas Pavlik, Jiri Litzman, Marcela Vlkova
Introduction: Previous studies showed that several lymphocyte abnormalities seen in the most frequent symptomatic immunoglobulin deficiency, common variable immunodeficiency (CVID), were also observed in a genetically related asymptomatic disorder - selective IgA deficiency (IgAD). In this study we searched for abnormalities in the differentiation stages of T cells as well as for similarities of these abnormalities in CVID and IgAD patients. Material and methods: Using flow cytometry in 80 patients with IgAD, 48 patients with CVID, and 80 control persons we determined T-lymphocyte subsets: both CD4 and CD8 were divided into the naïve CD45RO-CD27+, early differentiated CD45RO+CD27+, late differentiated CD45RO+CD27- and fully differentiated effector CD45RO-CD27- memory T cells, as well as Treg cells, defined as CD4+CD25highCD127low T cells...
2017: Central-European Journal of Immunology
https://www.readbyqxmd.com/read/29184600/-common-variable-immune-deficiency-lately-revealed-by-gastrointestinal-problems-about-a-case
#2
Fatima Ezzaitouni, Youssef Thiyfa, Mohamed Tahiri, Fouad Haddad, Wafaa Hliwa, Ahmed Bellabah, Wafaa Badre
Common Variable Immune Deficiency (CVID) is rare. It is a constitutional deficit of humoral immunity characterized by recurrent bacterial infections and by increased frequency of tumors, autoimmune or granulomatous diseases. Gastrointestinal manifestations are very variable and sometimes reveal common variable immune deficiency. We report the case of a 31-year old patient with a history of childhood recurrent respiratory infections complicated by bronchiectasis and with a 3-year history of recurrent glairy diarrhea...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/29180260/a-novel-de-novo-activating-mutation-in-stat3-identified-in-a-patient-with-common-variable-immunodeficiency-cvid
#3
Mark A Russell, Manuela Pigors, Maha E Houssen, Ania Manson, David Kelsell, Hilary Longhurst, Noel G Morgan
Common variable immunodeficiency (CVID) is characterised by repeated infection associated with primary acquired hypogammaglobulinemia. CVID frequently has a complex aetiology but, in certain cases, it has a monogenic cause. Recently, variants within the gene encoding the transcription factor STAT3 were implicated in monogenic CVID. Here, we describe a patient presenting with symptoms synonymous with CVID, who displayed reduced levels of IgG and IgA, repeated viral infections and multiple additional co-morbidities...
November 24, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/29156507/liver-transplantation-in-adults-with-liver-disease-due-to-common-variable-immunodeficiency-leads-to-early-recurrent-disease-and-poor-outcome
#4
Vian Azzu, Joshua E Elias, Adam Duckworth, Susan Davies, Rebecca Brais, Dinakantha S Kumararatne, Alexander E S Gimson, William J H Griffiths
Common variable immunodeficiency (CVID) is the commonest from of primary immunodeficiency characterized by antibody deficiency, recurrent bacterial infections and autoimmunity. Advanced chronic liver disease occurs in a subset of patients with CVID and manifests with various histological features such as nodular regenerative hyperplasia, inflammation, fibrosis and cholangiopathy. We present a case series characterizing the outcomes in adult patients transplanted for primary CVID-related liver disease. We discuss the unique transplantation challenges faced in this primary immunodeficiency group including susceptibility to infections and early disease recurrence...
November 20, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/29138951/chronic-diarrhea-in-common-variable-immunodeficiency-a-case-series-and-review-of-the-literature
#5
Antonio Pecoraro, Liliana Nappi, Ludovica Crescenzi, Francesco P D'Armiento, Arturo Genovese, Giuseppe Spadaro
Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by reduced immunoglobulin serum levels and absent or impaired antibody production. Clinical manifestations, including infections, inflammatory and autoimmune diseases, and malignancies, also involve various segments of the gastrointestinal tract. Chronic diarrhea is one of the most common gastrointestinal symptoms and may cause a wide spectrum of potentially life-threatening conditions as malabsorption and protein-energy malnutrition...
November 14, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/29114494/giardiasis-mimicking-celiac-disease-in-a-patient-of-common-variable-immunodeficiency
#6
Kumar Saurabh, Vijaya Lakshmi Nag, Daisy Khera, Poonam Elhence
An adolescent boy presented to pediatrics outpatient department with complaints of recurrent diarrhea, nausea, vomiting, and pedal edema since 3-4 months, with no relief even after taking treatment. His investigation revealed decreased serum IgA, IgG, and IgM levels with hypoproteinemia. Duodenal biopsy showed features of celiac disease (CD), but tissue transglutaminase IgA was negative. In stool examination, plenty of Giardia lamblia cysts and eggs of Hymenolepis nana were present. He improved on treatment and remained asymptomatic for 4 months...
July 2017: Tropical Parasitology
https://www.readbyqxmd.com/read/29114388/epistatic-interactions-between-mutations-of-taci-tnfrsf13b-and-tcf3-result-in-a-severe-primary-immunodeficiency-disorder-and-systemic-lupus-erythematosus
#7
Rohan Ameratunga, Wikke Koopmans, See-Tarn Woon, Euphemia Leung, Klaus Lehnert, Charlotte A Slade, Jessica C Tempany, Anselm Enders, Richard Steele, Peter Browett, Philip D Hodgkin, Vanessa L Bryant
Common variable immunodeficiency disorders (CVID) are a group of primary immunodeficiencies where monogenetic causes account for only a fraction of cases. On this evidence, CVID is potentially polygenic and epistatic although there are, as yet, no examples to support this hypothesis. We have identified a non-consanguineous family, who carry the C104R (c.310T>C) mutation of the Transmembrane Activator Calcium-modulator and cyclophilin ligand Interactor (TACI, TNFRSF13B) gene. Variants in TNFRSF13B/TACI are identified in up to 10% of CVID patients, and are associated with, but not solely causative of CVID...
October 2017: Clinical & Translational Immunology
https://www.readbyqxmd.com/read/29103189/modulation-of-the-interleukin-21-pathway-with-interleukin-4-distinguishes-common-variable-immunodeficiency-patients-with-more-non-infectious-clinical-complications
#8
Marylin Desjardins, Marianne Béland, Marieme Dembele, Duncan Lejtenyi, Jean-Phillipe Drolet, Martine Lemire, Christos Tsoukas, Moshe Ben-Shoshan, Francisco J D Noya, Reza Alizadehfar, Christine T McCusker, Bruce D Mazer
PURPOSE: Common variable immunodeficiency (CVID) is characterized by hypogammaglobulinemia and clinical manifestations such as infections, autoimmunity, and malignancy. We sought to determine if responsiveness to interleukin-21 (IL-21), a key cytokine for B cell differentiation, correlates with distinct clinical phenotypes in CVID. METHODS: CVID subjects were recruited through the Canadian Primary Immunodeficiency Evaluative Survey registry. Peripheral blood mononuclear cells were cultured with anti-CD40 ± interferon-gamma, interleukin-4 (IL-4), IL-21, and/or IL-4+IL-21...
November 4, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/29083052/skewed-invariant-natural-killer-t-inkt-cells-impaired-inkt-b-cell-help-and-decreased-sap-expression-in-blood-lymphocytes-from-patients-with-common-variable-immunodeficiency
#9
L V Erazo-Borrás, J A Álvarez-Álvarez, C A Perez-Romero, J C Orrego-Arango, J L Franco-Restrepo, C M Trujillo-Vargas
Common variable immunodeficiency (CVID) is a syndrome with predominantly defective B cell function. However, abnormalities in the number and function of other lymphocyte subpopulations in peripheral blood (PB) have been described in most patients. We have analysed the distribution of iNKT cell subpopulations in the PB of CVID patients and the ability of these cells to provide in vitro cognate B cell help. The total of iNKT cells was reduced in the PB of CVID patients, especially CD4+, CD4-/CD8- and CCR5+/CXCR3+...
September 2017: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/29080979/autoimmune-cytopenias-and-associated-conditions-in-cvid-a-report-from-the-usidnet-registry
#10
Elizabeth J Feuille, Niloofar Anooshiravani, Kathleen E Sullivan, Ramsay L Fuleihan, Charlotte Cunningham-Rundles
PURPOSE: Autoimmune cytopenia is frequently a presenting manifestation of common variable immune deficiency (CVID). Studies characterizing the CVID phenotype associated with autoimmune cytopenias have mostly been limited to large referral centers. Here, we report prevalence of autoimmune cytopenias in CVID from the USIDNET Registry and compare the demographics and clinical features of patients with and without this complication. METHODS: Investigators obtained demographic, laboratory, and clinical data on CVID patients within the USIDNET Registry...
October 28, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/29033928/unexpectedly-high-prevalence-of-common-variable-immunodeficiency-in-finland
#11
Jannica S Selenius, Timi Martelius, Sampsa Pikkarainen, Sanna Siitonen, Eero Mattila, Risto Pietikäinen, Pekka Suomalainen, Arja H Aalto, Janna Saarela, Elisabet Einarsdottir, Asko Järvinen, Martti Färkkilä, Juha Kere, Mikko Seppänen
BACKGROUND: Common variable immunodeficiency (CVID) is the most common primary immunodeficiency. Prevalence varies greatly between countries and studies. Most diagnostic criteria include hypogammaglobulinemia and impaired vaccine response. AIM: To evaluate the minimum prevalence as well as the clinical and immunological phenotypes of CVID in Southern Finland. METHODS: We performed a cross-sectional study to assess all adult CVID patients followed up in three hospital districts in Southern and South-Eastern Finland between April 2007 and August 2015...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/29030829/review-diagnosing-common-variable-immunodeficiency-disorder-in-the-era-of-genome-sequencing
#12
REVIEW
Rohan Ameratunga, Klaus Lehnert, See-Tarn Woon, David Gillis, Vanessa L Bryant, Charlotte A Slade, Richard Steele
Common variable immunodeficiency disorders (CVID) are an enigmatic group of often heritable conditions, which may manifest for the first time in early childhood or as late as the eighth decade of life. In the last 5 years, next generation sequencing (NGS) has revolutionised identification of genetic disorders. However, despite the best efforts of researchers around the globe, CVID conditions have been slow to yield their molecular secrets. We have previously described the many clinical advantages of identifying the genetic basis of primary immunodeficiency disorders (PIDs)...
October 14, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/29030367/patients-with-common-variable-immunodeficiency-paradoxically-have-increased-rates-of-autoimmune-disorders
#13
Ammu Thampi Susheela, Andrew Hale
Common variable immunodeficiency (CVID), characterised by disordered B cell function, is one of the most common primary immunodeficiency disorders. Patients with CVID are at lifelong risk of recurrent infections, particularly of the respiratory and gastrointestinal tracts. Paradoxically, given their immunocompromised state, patients with CVID are also at significantly increased risk of autoimmune disorders, which are seen in almost 25% of cases. The authors report a 24-year-old female patient with CVID, manifested as severe hypogammaglobulinaemia with recurrent sinopulmonary infections and enterocolitis, who presented with transaminitis, chronic diarrhoea and haematemesis...
October 13, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28983810/dysregulation-of-innate-lymphoid-cells-in-common-variable-immunodeficiency
#14
REVIEW
Paul J Maglione, Montserrat Cols, Charlotte Cunningham-Rundles
Common variable immunodeficiency (CVID) is the most prevalent symptomatic primary immune deficiency. With widespread use of immunoglobulin replacement therapy, non-infectious complications, such as autoimmunity, chronic intestinal inflammation, and lung disease, have replaced infections as the major cause of morbidity and mortality in this immune deficiency. The pathogenic mechanisms that underlie the development of these complications in CVID are not known; however, there have been numerous associated laboratory findings...
October 5, 2017: Current Allergy and Asthma Reports
https://www.readbyqxmd.com/read/28983794/somatic-hypermutation-defects-in-common-variable-immune-deficiency
#15
REVIEW
María Belén Almejun, Mercedes Borge
Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by impaired antibody production and recurrent infections. In the last 20 years, several groups have reported that B cells from CVID patients have an impaired somatic hypermutation (SHM). The reported frequency of this defect among CVID patient cohorts is highly variable and so is the methodology used to evaluate this process. Interestingly, the low level of SHM on B cells from CVID patients has been correlated with the presence of infectious and non-infectious complications...
October 5, 2017: Current Allergy and Asthma Reports
https://www.readbyqxmd.com/read/28983790/influenza-vaccination-in-patients-with-common-variable-immunodeficiency-cvid
#16
REVIEW
Jan F Mieves, Kirsten Wittke, Helma Freitag, Hans-Dieter Volk, Carmen Scheibenbogen, Leif G Hanitsch
PURPOSE OF REVIEW: Vaccination against influenza in patients with primary antibody deficiency is recommended. Common variable immunodeficiency (CVID) is the most frequent and clinically relevant antibody deficiency disease and is by definition characterized by an impaired vaccination response. The purpose of this review is to present the current knowledge of humoral and cellular vaccine response to influenza in CVID patients. RECENT FINDINGS: Studies conducted in CVID patients demonstrated an impaired humoral response upon influenza vaccination...
October 5, 2017: Current Allergy and Asthma Reports
https://www.readbyqxmd.com/read/28973009/tlr9-stimulation-of-b-cells-induces-transcription-of-p53-and-prevents-spontaneous-and-irradiation-induced-cell-death-independent-of-dna-damage-responses-implications-for-common-variable-immunodeficiency
#17
Kristine Lillebø Holm, Randi Gussgard Syljuåsen, Grete Hasvold, Lene Alsøe, Hilde Nilsen, Kristina Ivanauskiene, Philippe Collas, Sergey Shaposhnikov, Andrew Collins, Randi Larsen Indrevær, Pål Aukrust, Børre Fevang, Heidi Kiil Blomhoff
In the present study, we address the important issue of whether B-cells protected from irradiation-induced cell death, may survive with elevated levels of DNA damage. If so, such cells would be at higher risk of gaining mutations and undergoing malignant transformation. We show that stimulation of B-cells with the TLR9 ligands CpG-oligodeoxynucleotides (CpG-ODN) prevents spontaneous and irradiation-induced death of normal peripheral blood B-cells, and of B-cells from patients diagnosed with Common variable immunodeficiency (CVID)...
2017: PloS One
https://www.readbyqxmd.com/read/28937520/evans-syndrome-as-first-manifestation-of-primary-immunodeficiency-in-clinical-practice
#18
Libny Martínez-Valdez, Angela Deyà-Martínez, María T Giner, Rubén Berrueco, Ana Esteve-Solé, Manel Juan, Ana M Plaza-Martín, Laia Alsina
BACKGROUND: Evans syndrome (ES) is a rare immune disorder in children, manifested by simultaneous or sequential autoimmune cytopenias (ACs) of unknown cause and having a chronic course with periods of exacerbation and remission. Some primary immunodeficiencies (PIDs) may present with autoimmune manifestations without infections, masking suspicion of them. The PIDs that can typically manifest as ES are autoimmune lymphoproliferative syndrome and common variable immunodeficiency (CVID)...
October 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28936778/preferential-reduction-of-circulating-innate-lymphoid-cells-type-2-in-patients-with-common-variable-immunodeficiency-with-secondary-complications-is-part-of-a-broader-immune-dysregulation
#19
David Friedmann, Baerbel Keller, Ina Harder, Jonas Schupp, Yakup Tanriver, Susanne Unger, Klaus Warnatz
PURPOSE: Over a third of patients with common variable immunodeficiency (CVID) suffer from secondary complications like inflammatory organ disease, autoimmune manifestations, or lymphoproliferation contributing to increased morbidity and mortality in affected patients. Innate lymphoid cells (ILCs) have emerging roles in setting the milieu for physiological, but also pathological, immune responses and inflammation. We therefore sought to correlate the recently identified disturbed homeostasis of ILCs with alterations of the adaptive immune system in complex CVID patients (CVIDc)...
November 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28929596/hypogammaglobulinemia-in-children-a-warning-sign-to-look-deeply
#20
Karina Mescouto de Melo, Maria Isabel de Moraes-Pinto, Luís E C Andrade, Reinaldo Salomão, Milena K C Brunialti, Vanessa S Ferreira, Beatriz T Costa-Carvalho
This study investigated phenotypic and functional characteristics of lymphocytes in children with common variable immunodeficiency (CVID) and unclassified hypogammaglobulinemia (UH), as well as B-cell subsets in non-consanguineous parents. Blood samples of 30 children, CVID (n = 9), UH (n = 9), healthy donors HD (n = 12), and 19 adults (parents and controls) were labeled by a combination of surface markers to identify CD4, CD8 T-cell and B-cell subpopulations. T-cell cytokine production in children was analyzed in vitro after stimulation with phytohemagglutinin (PHA) and tetanus toxoid...
October 2017: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
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