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    Acute Rejection of a Kidney Transplant in a Patient With Common Variable Immunodeficiency: A Case Report.
    Transplant Proc 2017 Mar;49(2):380-385
    Department of Medicine, Division of Nephrology and Hypertension, Vanderbilt University Medical Center, Nashville, Tennessee, USA. Electronic address:
    Common variable immunodeficiency is a primary immunodeficiency characterized by hypogammaglobulinemia and recurrent bacterial infections. We report a case of a 44-year-old male patient with end-stage renal disease and an established diagnosis of common variable immunodeficiency who underwent a living unrelated kidney transplant. He remained nearly infection free on maintenance immunoglobulin replacement. Read More

    Human NLRP3 inflammasome activity is regulated by and potentially targetable via BTK.
    J Allergy Clin Immunol 2017 Feb 16. Epub 2017 Feb 16.
    Interfaculty Institute for Cell Biology, Department of Immunology, University of Tübingen, Auf der Morgenstelle 15, 72076 Tübingen, Germany. Electronic address:
    Background: The Nod-like receptor, NACHT, LRR and PYD domains-containing protein 3 (NLRP3), and Bruton's tyrosine kinase (BTK) are protagonists in innate and adaptive immunity, respectively: NLRP3 senses exogenous and endogenous insults leading to inflammasome activation, which occurs spontaneously in Muckle-Wells Syndrome (MWS); BTK mutations cause the genetic immunodeficiency X-linked agammaglobulinemia (XLA). However, to date few proteins that regulate NLRP3 inflammasome activity in human primary immune cells have been identified and clinically promising pharmacological targeting strategies remain elusive.

    Objective: We therefore sought to identify novel regulators of the NLRP3 inflammasome in human cells with a view to exploring interference with inflammasome activity at the level of such regulators. Read More

    Diagnosis of primary antibody and complement immunodeficiencies in young adults after a first invasive bacterial infection.
    Clin Microbiol Infect 2017 Feb 10. Epub 2017 Feb 10.
    Univ. Lille, INSERM U995 - LIRIC - Lille Inflammation Research International Center, F-59000 Lille, France; CHU Lille, Département de Médecine Interne et Immunologie Clinique, Centre National de Référence Maladies Systémiques et Auto-immunes Rares, F-59000 Lille, France; CHU Lille, Institut d'Immunologie, F-59000 Lille, France. Electronic address:
    Objectives: Screening for primary immunodeficiencies (PIDs) in adults is recommended after two severe bacterial infections. We aimed to evaluate if screening should be performed after the first invasive infection in young adults.

    Methods: Eligible patients were retrospectively identified using hospital discharge and bacteriology databases in 3 centers during a 3-year period. Read More

    LRRC8A-dependent volume regulated anion channel activity is dispensable for T cell development and function.
    J Allergy Clin Immunol 2017 Feb 9. Epub 2017 Feb 9.
    Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, MA. Electronic address:
    Background: Leucine-rich repeat containing 8A (LRRC8A) is a ubiquitously expressed transmembrane protein with 17 LRRs at its C-terminal end, and is an essential component of the volume regulated anion channel (VRAC) which controls cellular volume. A heterozygous mutation in LRRC8A that truncates the two terminal LRRs was reported in a patient with agammaglobulinemia and absent B cells, and was demonstrated to exert a dominant negative effect on T and B cell development in mice. Lrrc8a(-/-) mice have severely defective T cell development and function. Read More

    [Immunological alterations in common variable immunodeficiency].
    Rev Alerg Mex 2017 Jan-Mar;64(4):87-108
    Secretaría de Salud, Instituto Nacional de Pediatría, Unidad de Investigación en Inmunodeficiencias. Ciudad de México, México.
    Common variable immunodeficiency (CVID) is the largest group of symptomatic primary immune deficiencies; it is characterized by hypogammaglobulinemia, poor response to vaccines and increased susceptibility to infections. Cellular phenotypes and abnormalities have been described both in adaptive and innate immune response. Several classifications of common variable immunodeficiency are based on defects found on T and B cells, which have been correlated with clinical manifestations. Read More

    Ibrutinib-associated bleeding: pathogenesis, management, and risk reduction strategies.
    J Thromb Haemost 2017 Feb 9. Epub 2017 Feb 9.
    Division of Hematology and Medical Oncology, Oregon Health & Science University, Knight Cancer Institute, Portland, Oregon.
    Ibrutinib is an irreversible inhibitor of Bruton's tyrosine kinase (Btk) that has proven to be an effective therapeutic agent for multiple B-cell mediated lymphoproliferative disorders. Ibrutinib, however, carries an increased bleeding risk compared to standard chemotherapy. Bleeding events range from minor mucocutaneous bleeding to life-threatening hemorrhage, due in large part to the effects of ibrutinib on several distinct platelet signaling pathways. Read More

    Inflammatory Duodenal Polyposis Associated with Primary Immunodeficiency Disease: A Novel Case Report.
    Case Rep Med 2017 10;2017:6206085. Epub 2017 Jan 10.
    Department of Sports Medicine, Safdarjung Hospital, New Delhi, India.
    Agammaglobulinemia is a rare form of B-cell primary immunodeficiency disease characterized by reduced levels of IgG, IgA, or IgM and recurrent bacterial infections. Agammaglobulinemia is most commonly associated with diffuse nodular lymphoid hyperplasia. Duodenal polyps are a rare entity; however, due to wide use of esophagogastroduodenoscopy, incidental diagnosis of duodenal polyps appears to be increasing. Read More

    The Clinical Significance of Complete Class Switching Defect in Ataxia Telangiectasia Patients.
    Expert Rev Clin Immunol 2017 Feb 4. Epub 2017 Feb 4.
    a Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran.
    Objectives: Ataxia telangiectasia (AT) is a primary immunodeficiency associated with recurrent infections. We aimed to investigate clinical and immunological classification in AT patients who suffer from a different spectrum of humoral immune defects.

    Methods: AT patients were categorized according to the ability of class switching and patients with hyper IgM (HIgM) profile were defined as class switching defect (CSD). Read More

    Ataxia-telangiectasia: Immunodeficiency and survival.
    Clin Immunol 2017 Jan 24. Epub 2017 Jan 24.
    Department of Neurology - Pediatric Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.
    Ataxia-telangiectasia (AT) is a neurodegenerative disorder characterized by ataxia, telangiectasia, and immunodeficiency. An increased risk of malignancies and respiratory diseases dramatically reduce life expectancy. To better counsel families, develop individual follow-up programs, and select patients for therapeutic trials, more knowledge is needed on factors influencing survival. Read More

    Damaging heterozygous mutations in NFKB1 lead to diverse immunological phenotypes.
    J Allergy Clin Immunol 2017 Jan 20. Epub 2017 Jan 20.
    Adult Immunodeficiency Unit, Infectious Diseases, Inflammation Center, University of Helsinki and Helsinki University Hospital, Helsinki, Finland; Rare Diseases Center, Children's Hospital, University of Helsinki and Helsinki University Hospital, P.O.Box 280, FI-00029 HUS, Finland.
    Background: The NF-κB signaling pathway is a key regulator of immune responses. Accordingly, mutations in several NF-κB pathway genes cause immunodeficiency.

    Objective: We sought to identify the cause of disease in three unrelated Finnish kindreds with variable symptoms of immunodeficiency and autoinflammation. Read More

    Pattern recognitions receptors in immunodeficiency disorders.
    Eur J Pharmacol 2017 Jan 14. Epub 2017 Jan 14.
    Mycobacteriology Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran.
    Pattern recognition receptors (PRRs) recognize common microbial or host-derived macromolecules and have important roles in early activation and response of the immune system. Initiation of the innate immune response starts with the recognition of microbial structures called pathogen associated molecular patterns (PAMPs). Recognition of PAMPs is performed by germline-encoded receptors expressed mainly on immune cells termed pattern recognition receptors (PRRs). Read More

    Thymoma-associated immunodeficiency: a diagnostic challenge for the clinician.
    Asian Cardiovasc Thorac Ann 2017 Feb 9;25(2):146-149. Epub 2017 Jan 9.
    1 Department of Respiratory Medicine, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India.
    Good's syndrome or thymoma-associated immunodeficiency is a rare clinical entity that is often presumed to be common variable immunodeficiency, due to lack of awareness and recognition of this syndrome. This syndrome more often goes unrecognized if a thymoma is not detected. An appropriate immunological work-up that aids timely diagnosis and adequate therapy with antimicrobials and intravenous immunoglobulins are mandatory to prevent the long-term complications and mortality associated with this syndrome. Read More

    The effect of continuous B cell depletion with rituximab on pathogenic autoantibodies and total IgG levels in ANCA vasculitis.
    Arthritis Rheumatol 2016 Dec 28. Epub 2016 Dec 28.
    Vasculitis and Glomerulonephritis Center, Division of Nephrology, Massachusetts General Hospital, Boston, MA.
    Objective: To evaluate the effect of rituximab on pathogenic autoantibodies and total immunoglobulin levels, and to identify serious adverse events, in patients with ANCA vasculitis treated with continuous B cell depletion.

    Methods: We conducted a retrospective analysis of 239 patients with ANCA vasculitis treated with rituximab-induced continuous B cell depletion. Two treatment cohorts were analyzed: an induction group (n= 53) and a maintenance group (n=237). Read More

    Major infections, secondary cancers and autoimmune diseases occur in different clinical subsets of chronic lymphocytic leukaemia patients.
    Eur J Cancer 2017 Feb 24;72:103-111. Epub 2016 Dec 24.
    Hematology and Clinical Immunology Unit, Department of Medicine, University of Padua, Italy; Venetian Institute of Molecular Medicine, Centro di Eccellenza per la Ricerca Biomedica Avanzata, Italy. Electronic address:
    Background: Major infections (MIs), secondary cancers (SCs) and autoimmune diseases (ADs) are the most common and relevant complications in patients with chronic lymphocytic leukaemia.

    Methods: We performed a single-centre retrospective study to investigate the prevalence of the above quoted complications, the association with most important prognostic markers and their impact on survival (n = 795).

    Results: Almost one out of three patients experienced at least one complication and only 0. Read More

    A novel BTK gene mutation, c.82delC (p.Arg28 Alafs(*)5), in a Korean family with X-linked agammaglobulinemia.
    Korean J Pediatr 2016 Nov 30;59(Suppl 1):S49-S52. Epub 2016 Nov 30.
    Department of Pediatrics, Soonchunhyang University Seoul Hospital, Soonchunhyang University College of Medicine, Seoul, Korea.; Pediatric Allergy and Respiratory Center, Soonchunhyang University Seoul Hospital, Soonchunhyang University College of Medicine, Seoul, Korea.
    X-linked agammaglobulinemia (XLA) is a hereditary humoral immunodeficiency that results from Bruton's tyrosine kinase (BTK) gene mutations. These mutations cause defects in B-cell development, resulting in the virtual absence of these lymphocytes from the peripheral circulation. Consequently, this absence leads to a profound deficiency of lg all isotypes, and an increased susceptibility to encapsulated bacterial infections. Read More

    Autoimmunity in Primary Antibody Deficiencies.
    Int Arch Allergy Immunol 2016 24;171(3-4):180-193. Epub 2016 Dec 24.
    Department of Laboratory Medicine, Imam Hassan Mojtaba Hospital, Alborz University of Medical Sciences, Karaj, Iran.
    Primary antibody deficiencies (PADs) are the most common inherited primary immunodeficiencies in humans, characterized by hypogammaglobulinemia, an inability to produce specific antibodies, and recurrent infections mainly caused by encapsulated bacteria. However, it has been shown that inflammatory disorders, granulomatous lesions, lymphoproliferative diseases, cancer, and autoimmunity are associated with the various types of PAD. Both systemic and organ-specific autoimmune diseases could be attributed to B-cell defects in PAD patients. Read More

    Combined immunodeficiency and Epstein-Barr virus-induced B cell malignancy in humans with inherited CD70 deficiency.
    J Exp Med 2017 Jan 23;214(1):91-106. Epub 2016 Dec 23.
    Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institutet at Karolinska University Hospital Huddinge, SE1418 Stockholm, Sweden
    In this study, we describe four patients from two unrelated families of different ethnicities with a primary immunodeficiency, predominantly manifesting as susceptibility to Epstein-Barr virus (EBV)-related diseases. Three patients presented with EBV-associated Hodgkin's lymphoma and hypogammaglobulinemia; one also had severe varicella infection. The fourth had viral encephalitis during infancy. Read More

    Comprehensive genetic testing for primary immunodeficiency disorders in a tertiary hospital: 10-year experience in Auckland, New Zealand.
    Allergy Asthma Clin Immunol 2016 7;12:65. Epub 2016 Dec 7.
    Department of Virology and Immunology, LabPLUS, Auckland City Hospital, Grafton, Auckland, 1148 New Zealand.
    Background And Purpose: New Zealand is a developed geographically isolated country in the South Pacific with a population of 4.4 million. Genetic diagnosis is the standard of care for most patients with primary immunodeficiency disorders (PIDs). Read More

    Secondary antibody deficiency - causes and approach to diagnosis.
    Clin Med (Lond) 2016 Dec;16(6):571-576
    Department of Clinical Immunology and Allergy, St James's University Hospital, Leeds, UK.
    Antibody deficiencies can occur in the context of primary disorders due to inherited genetic defects; however, secondary immune disorders are far more prevalent and can be caused by various diseases and their treatment, certain medications and surgical procedures. Immunoglobulin replacement therapy has been shown to be effective in reducing infections, morbidity and mortality in primary antibody deficiencies but secondary antibody deficiencies are in general poorly defined and there are no guidelines for the management of patients with this condition. Clinical decisions are based on experience from primary antibody deficiencies. Read More

    Patterns of constitutively phosphorylated kinases in B cells are associated with disease severity in common variable immunodeficiency.
    Clin Immunol 2017 Feb 3;175:69-74. Epub 2016 Dec 3.
    K.G. Jebsen Centre for Cancer Immunotherapy, Institute of Clinical Medicine, University of Oslo, Oslo, Norway; K.G. Jebsen Inflammation Research Centre, Institute of Clinical Medicine, University of Oslo, Oslo, Norway; Research Institute of Internal Medicine, Oslo University Hospital, Rikshospitalet, Oslo, Norway; Section of Clinical Immunology and Infectious Diseases, Oslo University Hospital, Rikshospitalet, Oslo, Norway. Electronic address:
    Patients with common variable immunodeficiency (CVID) constitute a clinically and immunologically heterogeneous group characterized by B-cell dysfunction with hypogammaglobulinemia and defective immunoglobulin class switch of unknown etiology. Current classification systems are insufficient to achieve precise disease management. Characterization of signaling pathways essential for B-cell differentiation and class switch could provide new means to stratify patients. Read More

    The CXCL12/CXCR4 Signaling Pathway: A New Susceptibility Factor in Human Papillomavirus Pathogenesis.
    PLoS Pathog 2016 Dec 5;12(12):e1006039. Epub 2016 Dec 5.
    Inflammation Chemokines and Immunopathology, INSERM, Fac. de médecine-Univ Paris-Sud, Université Paris-Saclay, Clamart, France.
    The productive human papillomavirus (HPV) life cycle is tightly linked to the differentiation and cycling of keratinocytes. Deregulation of these processes and stimulation of cell proliferation by the action of viral oncoproteins and host cell factors underlies HPV-mediated carcinogenesis. Severe HPV infections characterize the wart, hypogammaglobulinemia, infection, and myelokathexis (WHIM) immunodeficiency syndrome, which is caused by gain-of-function mutations in the CXCR4 receptor for the CXCL12 chemokine, one of which is CXCR41013. Read More

    Succinyl-CoA synthetase (SUCLA2) deficiency in two siblings with impaired activity of other mitochondrial oxidative enzymes in skeletal muscle without mitochondrial DNA depletion.
    Mol Genet Metab 2016 Nov 12. Epub 2016 Nov 12.
    Division of Genetics and Genomics, The Manton Center for Orphan Disease Research, Boston Children's Hospital, Boston, MA, USA; Department of Pediatrics, Harvard Medical School, Boston, MA, USA. Electronic address:
    Mutations in SUCLA2 result in succinyl-CoA ligase (ATP-forming) or succinyl-CoA synthetase (ADP-forming) (A-SCS) deficiency, a mitochondrial tricarboxylic acid cycle disorder. The phenotype associated with this gene defect is largely encephalomyopathy. We describe two siblings compound heterozygous for SUCLA2 mutations, c. Read More

    LPS-Responsive Beige-Like Anchor Gene Mutation Associated With Possible Bronchiolitis Obliterans Organizing Pneumonia Associated With Hypogammaglobulinemia and Normal IgM Phenotype and Low Number of B Cells.
    Acta Med Iran 2016 Oct;54(10):620-623
    Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. AND Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran. AND Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Boston, MA, USA.
    LPS-Responsive Beige-like Anchor (LRBA) deficiency is a disease which has recently been described in a group of patients with common variable immunodeficiency (CVID) in association with autoimmunity and/or inflammatory bowel disease (IBD)-like phenotype. We here describe a 10-year-old boy who experienced recurrent infections, mainly in the respiratory system, associated with thrombocytopenia and anemia. Immunological workup showed low numbers of B cells and low IgG, but normal IgM levels. Read More

    Newborn Screening for Severe Primary Immunodeficiency Diseases in Sweden-a 2-Year Pilot TREC and KREC Screening Study.
    J Clin Immunol 2017 Jan 21;37(1):51-60. Epub 2016 Nov 21.
    Department of Clinical Immunology, Karolinska University Hospital Huddinge, SE-14186, Stockholm, Sweden.
    Newborn screening for severe primary immunodeficiencies (PID), characterized by T and/or B cell lymphopenia, was carried out in a pilot program in the Stockholm County, Sweden, over a 2-year period, encompassing 58,834 children. T cell receptor excision circles (TREC) and kappa-deleting recombination excision circles (KREC) were measured simultaneously using a quantitative PCR-based method on DNA extracted from dried blood spots (DBS), with beta-actin serving as a quality control for DNA quantity. Diagnostic cutoff levels enabling identification of newborns with milder and reversible T and/or B cell lymphopenia were also evaluated. Read More

    [Sepsis due to Pseudomona as a debut of a primary immunodeficiency in a child].
    Arch Argent Pediatr 2016 Dec;114(6):e444-e447
    Hospital Universitario Miguel Servet, Zaragoza, España.
    X-linked agammaglobulinemia is a primary humoral immunodeficiency. It is a recessive X-linked disorder characterized by low or absent circulating mature B cells, hypo/agammaglobulinemia and no humoral response to immunizations due to mutations along chromosome X. It is characterized by severe, recurrent and difficult treatment infections. Read More

    Bruton's agammaglobulinemia in an adult male due to a novel mutation: a case report.
    J Thorac Dis 2016 Oct;8(10):E1207-E1212
    State Key Lab of Respiratory Disease, Guangzhou Institute of Respiratory Disease, The First Affiliated to Guangzhou Medical University, Guangzhou 510120, China; ; Guangzhou Institute of Biomedicine and Health, Chinese Academy of Sciences, Guangzhou 510530, China.
    X-linked agammaglobulinemia (XLA) is caused by mutation in the gene coding for Bruton's tyrosine kinase (BTK), which impairs peripheral B cell maturation and hypogammaglobulinemia. In this report, we present a case of XLA in a 22-year-old adult male. Genetic testing revealed a novel mutation located at the conserved region (c. Read More

    Evaluation of humoral immunity profiles to identify heart recipients at risk for development of severe infections: A multicenter prospective study.
    J Heart Lung Transplant 2016 Oct 17. Epub 2016 Oct 17.
    Clinical Immunology Department, Hospital General Universitario Gregorio Marañon, Madrid, Spain. Electronic address:
    Background: New biomarkers are necessary to improve detection of the risk of infection in heart transplantation. We performed a multicenter study to evaluate humoral immunity profiles that could better enable us to identify heart recipients at risk of severe infections.

    Methods: We prospectively analyzed 170 adult heart recipients at 8 centers in Spain. Read More

    Primary hypogammaglobulinemia: The impact of early diagnosis in lung complications.
    Rev Assoc Med Bras (1992) 2016 Sep;62(6):530-536
    Unit of Allergy and Immunology, Department of Pediatrics, Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo (HC-FMUSP), São Paulo, SP, Brazil.
    Objective:: To describe clinical features, tomographic findings and pulmonary function in pediatric patients with primary hypogammaglobulinemia (PH).

    Method:: A retrospective cohort study of children with PH who received intravenous immunoglobulin (IVIG) and prophylactic antibiotics between 2005 and 2010. Epidemiological and clinical features, computed tomography (CT) findings, and spirometric data were compared, assuming a 5% significance level. Read More

    Transient hypogammaglobulinaemia of infants in children with mastocytosis - strengthened indications for vaccinations.
    Cent Eur J Immunol 2016 25;41(3):282-286. Epub 2016 Oct 25.
    Department of Paediatrics, Haematology, and Oncology, Medical University of Gdansk, Poland.
    Mastocytosis is a disease caused by the accumulation of mast cells (MC) in the skin and/or in other tissues. Both the cutaneous form of the disease (CM) predominating in children and the systemic form (SM) typical for adults are associated with the occurrence of MC mediator-related symptoms. The release of mediators can be induced by physical stimuli and/or specific triggering factors. Read More

    Progressive severe B cell deficiency in pediatric Rubinstein-Taybi syndrome.
    Clin Immunol 2016 Dec 5;173:181-183. Epub 2016 Nov 5.
    Pediatrics Clinic and Institute for Molecular Medicine A. Nocivelli, Department of Clinical and Experimental Sciences, University of Brescia, Spedali Civili, Brescia, Italy.

    Immune Dysfunction After Completion of Childhood Leukemia Therapy.
    J Pediatr Hematol Oncol 2017 Jan;39(1):1-5
    *Children's Cancer and Blood Disorders Program, Children's Hospitals and Clinics of Minnesota †Pediatric Infectious Diseases and Immunology, Children's Hospitals and Clinics of Minnesota, Minneapolis, MN.
    Background: Children with leukemia suffer immune dysfunction from their malignancy and chemotherapy. The immune system components most affected, the degree to which immune suppression occurs, and the duration of immunodeficiency are incompletely characterized. This study measures immunologic parameters following completion of therapy. Read More

    Targeted antibody-mediated depletion of murine CD19 CAR T cells permanently reverses B cell aplasia.
    J Clin Invest 2016 Nov 17;126(11):4262-4272. Epub 2016 Oct 17.
    The adoptive transfer of T cells that have been genetically modified to express a CD19-specific chimeric antigen receptor (CAR) is effective for treating human B cell malignancies. However, the persistence of functional CD19 CAR T cells causes sustained depletion of endogenous CD19+ B cells and hypogammaglobulinemia. Thus, there is a need for a mechanism to ablate transferred T cells after tumor eradication is complete to allow recovery of normal B cells. Read More

    Deficiency of base excision repair enzyme NEIL3 drives increased predisposition to autoimmunity.
    J Clin Invest 2016 Nov 17;126(11):4219-4236. Epub 2016 Oct 17.
    Alterations in the apoptosis of immune cells have been associated with autoimmunity. Here, we have identified a homozygous missense mutation in the gene encoding the base excision repair enzyme Nei endonuclease VIII-like 3 (NEIL3) that abolished enzymatic activity in 3 siblings from a consanguineous family. The NEIL3 mutation was associated with fatal recurrent infections, severe autoimmunity, hypogammaglobulinemia, and impaired B cell function in these individuals. Read More

    Rituximab-Related Late-Onset Neutropenia in Kidney Transplant Recipients Treated for Antibody-Mediated Acute Rejection.
    Exp Clin Transplant 2016 Oct 31. Epub 2016 Oct 31.
    From the Faculty of Pharmacy, Tehran University of Medical Sciences, Tehran, Iran.
    Objectives: Kidney transplant is a new area for use of rituximab, which is being used to treat acute antibody-mediated rejection or as an induction agent in ABO- or HLA-incompatible grafts. We report on late-onset neutropenia in rituximab-treated kidney transplant recipients with antibody-mediated rejection.

    Materials And Methods: This observational prospective study was performed on kidney transplant recipients with clinically suspicious or biopsy-proven antibody-mediated rejection treated with plasmapheresis plus intravenous immunoglobulin with (cases) or without (controls) rituximab. Read More

    [Clinic of humoral primary immunodeficiencies in adults. Experience in a tertiary hospital].
    Rev Alerg Mex 2016 Oct-Dec;63(4):334-341
    Instituto Mexicano del Seguro Social, Centro Médico Nacional Siglo XXI, Hospital de Especialidades, Servicio de Alergia e Inmunología Clínica, Ciudad de México, México.
    Background: Primary immunodeficiencies (PID) are characterized by alteration of the components of the immune system. Humoral deficiencies represent 50%. The most common are selective IgA deficiency, Bruton agammaglobulinemia, and common variable immunodeficiency (CVID). Read More

    Post-transplant hypogammaglobulinemia and risk of infection after kidney transplantation: Magnitude matters.
    Transpl Infect Dis 2017 Feb 16;19(1). Epub 2016 Dec 16.
    Unit of Infectious Diseases, Hospital Universitario "12 de Octubre", Instituto de Investigación Hospital "12 de Octubre" (i+12), School of Medicine, Universidad Complutense, Madrid, Spain.

    Risks and Benefits of Rituximab in the Treatment of Hashimoto Encephalopathy in Children: Two Case Reports and a Mini Review.
    Pediatr Neurol 2017 Jan 20;66:28-31. Epub 2016 Sep 20.
    Department of Pediatric Immunology and Rheumatology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, Netherlands. Electronic address:
    Hashimoto encephalopathy is a rare condition, characterized by the association of encephalopathy with a variety of neurological symptoms and autoantibodies to the thyroid gland. Its etiology is unknown, and symptoms are usually treated with immune suppressive therapy, e.g. Read More

    Early intravenous immunoglobulin replacement in hypogammaglobulinemic heart transplant recipients: results of a clinical trial.
    Transpl Infect Dis 2016 Dec 25;18(6):832-843. Epub 2016 Oct 25.
    Transplant Immunology Group, Clinical Immunology Department, Hospital General Universitario Gregorio Marañon, Madrid, Spain.
    Background: Immunoglobulin G (IgG) hypogammaglobulinemia (HGG) is a risk factor for development of severe infections after heart transplantation. We performed a clinical trial to preliminarily evaluate the efficacy and safety of early administration of intravenous immunoglobulin (IVIG) for prevention of severe infection in heart recipients with post-transplant IgG HGG.

    Methods: Twelve heart recipients with IgG HGG detected in a screening phase of the clinical trial (IgG <500 mg/dL) were recruited. Read More

    Intravenous Immune Globulin Stewardship Program at a Tertiary Academic Medical Center.
    Ann Pharmacother 2017 Feb 6;51(2):135-139. Epub 2016 Oct 6.
    1 Brigham and Women's Hospital, Boston, MA, USA.
    Background: In October 2010, a pharmacist-driven stewardship program was implemented at the Brigham and Women's Hospital to ensure continued adherence to the prescribing guideline, focusing on indications for intravenous immune globulin (IVIG) use and dosing per ideal body weight.

    Objective: The primary objective was to describe an IVIG stewardship program at a tertiary academic medical center.

    Methods: This was a prospective, observational study from January 2013 through December 2014. Read More

    Ibrutinib inhibition of Bruton protein-tyrosine kinase (BTK) in the treatment of B cell neoplasms.
    Pharmacol Res 2016 Nov 15;113(Pt A):395-408. Epub 2016 Sep 15.
    Blue Ridge Institute for Medical Research, 3754 Brevard Road, Suite 116, Box 19, Horse Shoe, NC 28742-8814, United States. Electronic address:
    The Bruton non-receptor protein-tyrosine kinase (BTK), a deficiency of which leads to X-linked agammaglobulinemia, plays a central role in B cell antigen receptor signaling. Owing to the exclusivity of this enzyme in B cells, the acronym could represent B cell tyrosine kinase. BTK is activated by the Lyn and SYK protein kinases following activation of the B cell receptor. Read More

    Rituximab for Rheumatoid Arthritis.
    Rheumatol Ther 2015 Dec 19;2(2):99-111. Epub 2015 Aug 19.
    University of Toronto, Toronto, Canada.
    Rituximab is a chimeric monoclonal antibody directed at the CD20 molecule on the surfaces of some but not all B cells. It depletes almost all peripheral B cells, but other niches of B cells are variably depleted, including synovium. Its mechanism of action in rheumatoid arthritis (RA) is only partially understood. Read More

    A Novel Mutation in a Critical Region for the Methyl Donor Binding in DNMT3B Causes Immunodeficiency, Centromeric Instability, and Facial Anomalies Syndrome (ICF).
    J Clin Immunol 2016 Nov 12;36(8):801-809. Epub 2016 Oct 12.
    Pediatric Department A and the Immunology Service, Jeffrey Modell Foundation Center, affiliated to the Sackler Faculty of Medicine, Tel Aviv University, Tel-Hashomer, Ramat Gan, Israel.
    Purpose: Immunodeficiency, centromeric instability, and facial anomalies (ICF) syndrome is an extremely rare autosomal recessive disease. The immune phenotype is characterized by hypogammaglobulinemia in the presence of B cells. T cell lymphopenia also develops in some patients. Read More

    Chronic Hepatitis E Infection in a Persistently Immunosuppressed Patient Unable to Be Eliminated after Ribavirin Therapy.
    Intern Med 2016;55(19):2811-2817. Epub 2016 Oct 1.
    Department of Gastroenterology and Hepatology, Yokosuka Kyosai Hospital, Japan.
    Recent case reports have shown that hepatitis E virus (HEV) infection can cause chronic hepatitis in immunosuppressed or immunocompromised patients. A 37-year-old woman suffered from prolonged elevation of aminotransferases after chemotherapy for Burkitt's lymphoma and was diagnosed with chronic hepatitis E due to a transfusion during chemotherapy. After an 8-month administration of ribavirin, complete HEV clearance was not achieved, likely due to prolonged hypogammaglobulinemia. Read More

    Hypogammaglobulinemia in infants receiving chronic peritoneal dialysis.
    Pediatr Nephrol 2017 Mar 7;32(3):503-509. Epub 2016 Oct 7.
    Division of Nephrology, Children's Mercy Hospital, University of Missouri-Kansas City School of Medicine, 2401 Gillham Road, Kansas City, MO, 64108, USA.
    Background: Peritonitis is a severe complication of chronic peritoneal dialysis (CPD) in infants. Few studies have been conducted to evaluate the relationship between hypogammaglobulinemia and peritonitis risk, and the potential benefit of intravenous immunoglobulins (IVIG) therapy in infants receiving CPD.

    Methods: Patients aged 0-12 months at initiation of CPD between 1985 and 2012 were eligible for inclusion in this retrospective study. Read More

    Comparison of various classifications for patients with common variable immunodeficiency (CVID) using measurement of B-cell subsets.
    Allergol Immunopathol (Madr) 2016 Oct 4. Epub 2016 Oct 4.
    Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. Electronic address:
    Background: Common variable immunodeficiency (CVID) is a heterogeneous disease, characterised by hypogammaglobulinaemia leading to recurrent infections and various complications. The aim of this study was to classify CVID patients based on four known classifications (Paris, Freiburg, EUROclass, and B-cell patterns) by measurement of B-cell subsets and to assess the relation of each classification with clinical manifestations.

    Methods: We measured all B-cell subsets as both absolute count and percentage in 30 CVID patients and 30 healthy individuals using four-colour flow cytometry. Read More

    Asthma and Hypogammaglobulinemia: an Asthma Phenotype with Low Type 2 Inflammation.
    J Clin Immunol 2016 Nov 6;36(8):810-817. Epub 2016 Oct 6.
    CHRU Tours, Service de Pneumologie et explorations fonctionnelles respiratoires, Hôpital Bretonneau, 2 boulevard Tonnellé, 37044, F-37044, Tours Cedex, France.
    Purpose: Little is known about hypogammaglobulinemia (HGG) in asthma patients. No data are available on the characteristics of adult patients with asthma and HGG.

    Methods: We conducted a retrospective monocentric study between January 2006 and December 2012. Read More

    Agammaglobulinemia associated to nasal polyposis due to a hypomorphic RAG1 mutation in a 12 years old boy.
    Clin Immunol 2016 Dec 3;173:121-123. Epub 2016 Oct 3.
    Department of Systems Medicine, "University of Rome Tor Vergata", Rome, Italy; University Department of Pediatrics, Unit of Immune and Infectious Diseases, Childrens' Hospital Bambino Gesù, Italy. Electronic address:
    Recombination-activating gene (RAG) 1 and 2 mutations in humans cause T(-) B(-) NK(+) SCID and Omenn syndrome, but milder phenotypes associated with residual protein activity have been recently described. We report a male patient with a diagnosis of common variable immunodeficiency (CVID) born from non-consanguineous parents, whose immunological phenotype was characterized by severe reduction of B cells and agammaglobulinemia for which several candidate genes were excluded by targeted Sanger sequencing. Next Generation Sequencing revealed two compound heterozygous mutations in the RAG1 gene: the previously described p. Read More

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