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Hypogammaglobulinemia

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https://www.readbyqxmd.com/read/28929596/hypogammaglobulinemia-in-children-a-warning-sign-to-look-deeply
#1
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
https://www.readbyqxmd.com/read/28928741/cxcl12-mediates-aberrant-costimulation-of-b-lymphocytes-in-warts-hypogammaglobulinemia-infections-myelokathexis-immunodeficiency
#2
Giuliana Roselli, Elisa Martini, Vassilios Lougaris, Raffaele Badolato, Antonella Viola, Marinos Kallikourdis
The Warts, Hypogammaglobulinemia, Infections, Myelokathexis (WHIM) syndrome is an immunodeficiency caused by mutations in chemokine receptor CXCR4. WHIM patient adaptive immunity defects remain largely unexplained. We have previously shown that WHIM-mutant T cells form unstable immunological synapses, affecting T cell activation. Here, we show that, in WHIM patients and WHIM CXCR4 knock-in mice, B cells are more apoptosis prone. Intriguingly, WHIM-mutant B cells were also characterized by spontaneous activation...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28925897/role-of-apoptosis-in-the-pathogenesis-of-common-variable-immunodeficiency-cvid
#3
Mazdak Ganjalikhani-Hakemi, Reza Yazdani, Mohammad Esmaeili, Hassan Abolhassani, Wiliam Rae, Gholamreza Azizi, Majid Zaki Dizaji, Mohammadreza Shaghaghi, Faezeh Abbasi-Rad, Abbas Rezaei, Sanaz Afshar-Qasemloo, Saeed Mohammadi, Nima Rezaei, Asghar Aghamohammadi
BACKGROUND: Common variable immunodeficiency (CVID) is a heterogeneous immune deficiency characterized by hypogammaglobulinemia. Since B cell maturation and differentiation is defective in this disorder, we evaluated apoptosis in B cells of patients with CVID compared with healthy donors (HD). METHODS: Determination of peripheral blood B-cell subsets in CVID and HDs, was performed using flow cytometry. We compared total apoptosis, early apoptosis and late apoptosis/necrosis in unstimulated and stimulated B-cells of patients with CVID and HDs...
September 19, 2017: Endocrine, Metabolic & Immune Disorders Drug Targets
https://www.readbyqxmd.com/read/28924106/intravenous-immunoglobulin-monotherapy-for-granulomatous-lymphocytic-interstitial-lung-disease-in-common-variable-immunodeficiency
#4
Mizue Hasegawa, Fumikazu Sakai, Asako Okabayashi, Akitoshi Sato, Naoko Yokohori, Hideki Katsura, Chihiro Asano, Toshiko Kamata, Eitetsu Koh, Yasuo Sekine, Kenzo Hiroshima, Takashi Ogura, Tamiko Takemura
Common variable immunodeficiency (CVID) is a heterogeneous subset of immunodeficiency disorders. Recurrent bacterial infection is the main feature of CVID, but various non-infectious complications can occur. A 42-year-old woman presented with cough and abnormal chest X-ray shadows. Laboratory tests showed remarkable hypogammaglobulinemia. Computed tomography revealed multiple consolidation and nodules on the bilateral lung fields, systemic lymphadenopathy, and splenomegaly. A surgical lung biopsy specimen provided the final diagnosis of lymphoproliferative disease in CVID, which was grouped under the term granulomatous lymphocytic interstitial lung disease...
September 15, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28916432/rituximab-as-induction-therapy-in-eosinophilic-granulomatosis-with-polyangiitis-refractory-to-conventional-immunosuppressive-treatment-a-36-month-follow-up-analysis
#5
Jens Thiel, Arianna Troilo, Ulrich Salzer, Theresa Schleyer, Kirsten Halmschlag, Marta Rizzi, Natalie Frede, Ana Venhoff, Reinhard E Voll, Nils Venhoff
BACKGROUND: Rituximab (RTX) is approved for induction therapy of granulomatosis with polyangiitis and microscopic polyangiitis. In eosinophilic granulomatosis with polyangiitis (EGPA), organ-threatening manifestations are mainly treated with cyclophosphamide (CYC). RTX as treatment in EGPA has been described in small case series; however long-term data and the efficacy of RTX in EGPA refractory to CYC have not been reported yet. OBJECTIVES: To investigate the efficacy and safety of RTX and conventional immunosuppressive therapy with CYC in EGPA as induction therapy and during long-term follow-up...
September 12, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28915275/long-term-efficacy-and-safety-of-rituximab-in-igg4-related-disease-data-from-a-french-nationwide-study-of-thirty-three-patients
#6
Mikael Ebbo, Aurélie Grados, Maxime Samson, Matthieu Groh, Anderson Loundou, Aude Rigolet, Benjamin Terrier, Constance Guillaud, Clarisse Carra-Dallière, Frédéric Renou, Agnieszka Pozdzik, Pierre Labauge, Sylvain Palat, Jean-Marie Berthelot, Jean-Loup Pennaforte, Alain Wynckel, Céline Lebas, Noémie Le Gouellec, Thomas Quémeneur, Karine Dahan, Franck Carbonnel, Gaëlle Leroux, Antoinette Perlat, Alexis Mathian, Patrice Cacoub, Eric Hachulla, Nathalie Costedoat-Chalumeau, Jean-Robert Harlé, Nicolas Schleinitz
OBJECTIVES: To assess efficacy and safety of rituximab (RTX) as induction therapy, maintenance of remission and treatment of relapses in a cohort of IgG4-related disease (IgG4-RD) patients. METHODS: Nationwide retrospective multicenter study of IgG4-RD patients treated with at least one course of RTX. Clinical, biological and radiological response, relapse rate and drug tolerance were analyzed. Kaplan-Meier curves were plotted and risk factors for relapse studied with a Cox regression model...
2017: PloS One
https://www.readbyqxmd.com/read/28906322/the-effect-of-ophthalmic-artery-chemosurgery-on-immune-function-in-retinoblastoma-patients-a-single-institution-retrospective-analysis
#7
Cheryl Fischer, Mary Petriccione, Stephanie Vitolano, Edith Guarini, Mary Elizabeth Davis, Ira J Dunkel
BACKGROUND: Ophthalmic artery chemosurgery (OAC) is associated with grade 3 and 4 neutropenia, however the effect on T-cell number and function is unknown. The purpose of this retrospective review was to confirm that patients treated with OAC do not develop immunosuppression warranting Pneumocystis pneumonia prophylaxis. PROCEDURE: IRB approval was obtained for a single center retrospective review of immune function tests in retinoblastoma patients who received OAC...
September 12, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28867533/-primary-intestinal-lymphangiectasia-waldmann-s-disease
#8
S Vignes, J Bellanger
Primary intestinal lymphangiectasia (PIL), Waldmann's disease, is a rare disorder of unknown etiology characterized by dilated intestinal lacteals leading to lymph leakage into the small-bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. The main symptom is bilateral lower limb edema. Edema may be moderate to severe including pleural effusion, pericarditis or ascites...
August 31, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28865061/severe-enteropathy-and-hypogammaglobulinemia-complicating-refractory-mycobacterium-tuberculosis-complex-disseminated-disease-in-a-child-with-il-12r%C3%AE-1-deficiency
#9
Andrés Augusto Arias, Carlos M Perez-Velez, Julio César Orrego, Marcela Moncada-Velez, Jessica Lineth Rojas, Alejandra Wilches, Andrea Restrepo, Mónica Trujillo, Carlos Garcés, Catalina Arango-Ferreira, Natalia González, Carmen Oleaga-Quintas, Diana Fernández, Johana Marcela Isaza-Correa, Diego Eduardo Gongóra, Daniel Gonzalez-Loaiza, Juan Esteban Sierra, Jean Laurent Casanova, Jacinta Bustamante, José Luis Franco
PURPOSE: Mendelian susceptibility to mycobacterial disease is a rare clinical condition characterized by a predisposition to infectious diseases caused by poorly virulent mycobacteria. Other infections such as salmonellosis and candidiasis are also reported. The purpose of this article is to describe a young boy affected with various infectious diseases caused by Mycobacterium tuberculosis complex, Salmonella sp, Klebsiella pneumonie, Citrobacter sp., and Candida sp, complicated with severe enteropathy and transient hypogammaglobulinemia...
September 1, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28861081/14%C3%A2-years-after-discovery-clinical-follow-up-on-15-patients-with-inducible-co-stimulator-deficiency
#10
Johanna Schepp, Janet Chou, Andrea Skrabl-Baumgartner, Peter D Arkwright, Karin R Engelhardt, Sophie Hambleton, Tomohiro Morio, Ekkehard Röther, Klaus Warnatz, Raif Geha, Bodo Grimbacher
BACKGROUND: Inducible co-stimulator (ICOS) deficiency was the first monogenic defect reported to cause common variable immunodeficiency (CVID)-like disease in 2003. Since then, 16 patients have been reported worldwide with an increasing range of clinical phenotypes. OBJECTIVE: We sought to compare the clinical and immunological phenotype and provide clinical follow-up and therapeutic approaches for treating ICOS-deficient patients. METHODS: We describe the clinical and laboratory data of 15 patients with available clinical data...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28852483/immunoglobulin-levels-and-infection-risk-with-rituximab-induction-for-anti-neutrophil-cytoplasmic-antibody-associated-vasculitis
#11
Shivani Shah, Khushleen Jaggi, Keiko Greenberg, Duvuru Geetha
BACKGROUND: Rituximab (RTX), a B cell-depleting anti-CD20 monoclonal antibody, is approved for treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Low immunoglobulin (Ig) levels have been observed surrounding RTX treatment. The association between the degree of Ig deficiency and infection risk is unclear in AAV patients. METHODS: AAV patients treated with RTX for remission induction at a single center (2005-15) with serum Ig measurements were included...
August 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28842786/neutropenia-in-patients-with-common-variable-immunodeficiency-a-rare-event-associated-with-severe-outcome
#12
Aurélien Guffroy, Rachel Mourot-Cottet, Laurence Gérard, Vincent Gies, Chantal Lagresle, Aurore Pouliet, Patrick Nitschké, Sylvain Hanein, Boris Bienvenu, Valérie Chanet, Jean Donadieu, Martine Gardembas, Marina Karmochkine, Raphaele Nove-Josserand, Thierry Martin, Vincent Poindron, Pauline Soulas-Sprauel, Fréderic Rieux-Laucat, Claire Fieschi, Eric Oksenhendler, Isabelle André-Schmutz, Anne-Sophie Korganow
BACKGROUND: Common variable immunodeficiency (CVID) is characterized by infections and hypogammaglobulinemia. Neutropenia is rare during CVID. METHODS: The French DEFI study enrolled patients with primary hypogammaglobulinemia. Patients with CVID and neutropenia were retrospectively analyzed. RESULTS: Among 473 patients with CVID, 16 patients displayed neutropenia (lowest count [0-1400]*10(6)/L). Sex ratio (M/F) was 10/6. Five patients died during the follow-up (11 years) with an increased percentage of deaths compared to the whole DEFI group (31...
August 26, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28814356/pneumococcal-13-valent-conjugate-vaccine-administration-after-inferior-response-to-pneumococcal-vaccine
#13
Ratika Gupta, Chak Shui Wong, Luz Fonacier
BACKGROUND: The pneumococcal vaccine, a nonconjugated vaccine, may be used to evaluate the integrity of the humoral immune system. Those patients with an inferior response to a nonconjugated vaccine may be vaccinated with a conjugated vaccine, which elicits both a B- and T-cell response. OBJECTIVE: We evaluated the immunogenicity of a conjugated vaccine in patients with inferior responses to a nonconjugated vaccine. METHODS: This was an institutional review board approved retrospective study that involved 22 patients with suspected specific antibody deficiency who received a nonconjugated vaccine, followed by a conjugated vaccine...
September 1, 2017: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/28808370/a-rare-case-of-strongyloides-hyperinfection-from-hypogammaglobulinemia
#14
Paidi Ramakrishna Reddy, Sujith M Thomas, A Rajalakshmi, Deepak Vijayan, Muraleedharan Raman
Strongyloides infection can range from asymptomatic eosinophilia to disseminated disease. Common in tropical and subtropical nations, it can lead to hyperinfection, an autoinfection increasing parasitic burden in immunocompromised. Cell-mediated immunity is important in combating parasite infection. We present a case of Strongyloides hyperinfection in hypogammaglobulinemia which was refractory to conventional treatment but responded to immunoglobulin administration with complete resolution indicating role of humoral immunity also...
July 2017: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28803861/long-duration-complete-remissions-of-diffuse-large-b-cell-lymphoma-after-anti-cd19-chimeric-antigen-receptor-t%C3%A2-cell-therapy
#15
James N Kochenderfer, Robert P T Somerville, Tangying Lu, James C Yang, Richard M Sherry, Steven A Feldman, Lori McIntyre, Adrian Bot, John Rossi, Norris Lam, Steven A Rosenberg
T cells expressing anti-CD19 chimeric antigen receptors (CARs) can induce complete remissions (CRs) of diffuse large B cell lymphoma (DLBCL). The long-term durability of these remissions is unknown. We administered anti-CD19 CAR T cells preceded by cyclophosphamide and fludarabine conditioning chemotherapy to patients with relapsed DLBCL. Five of the seven evaluable patients obtained CRs. Four of the five CRs had long-term durability with durations of remission of 56, 51, 44, and 38 months; to date, none of these four cases of lymphomas have relapsed...
July 13, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/28800262/risk-factors-predisposing-to-the-development-of-hypogammaglobulinemia-and-infections-post-rituximab
#16
Evangelos A A Christou, Giuliana Giardino, Austen Worth, Fani Ladomenou
Rituximab (RTX) is a monoclonal antibody against CD20, commonly used in the treatment of hematological malignancies and autoimmune diseases. The use of RTX is related to the development of hypogammaglobulinemia and infections. Aim of this review is to summarize the evidence supporting the association of specific risk factors with the development of hypogammaglobulinemia and infections post-RTX. Immunological complications are more common in patients with malignant diseases as compared to non-malignant diseases...
August 11, 2017: International Reviews of Immunology
https://www.readbyqxmd.com/read/28782633/plasma-cell-deficiency-in-human-subjects-with-heterozygous-mutations-in-sec61-translocon-alpha-1-subunit-sec61a1
#17
Desirée Schubert, Marie-Christine Klein, Sarah Hassdenteufel, Andrés Caballero-Oteyza, Linlin Yang, Michele Proietti, Alla Bulashevska, Janine Kemming, Johannes Kühn, Sandra Winzer, Stephan Rusch, Manfred Fliegauf, Alejandro A Schäffer, Stefan Pfeffer, Roger Geiger, Adolfo Cavalié, Hongzhi Cao, Fang Yang, Yong Li, Marta Rizzi, Hermann Eibel, Robin Kobbe, Amy L Marks, Brian P Peppers, Robert W Hostoffer, Jennifer M Puck, Richard Zimmermann, Bodo Grimbacher
BACKGROUND: Primary antibody deficiencies (PADs) are the most frequent primary immunodeficiencies in human subjects. The genetic causes of PADs are largely unknown. Sec61 translocon alpha 1 subunit (SEC61A1) is the major subunit of the Sec61 complex, which is the main polypeptide-conducting channel in the endoplasmic reticulum membrane. SEC61A1 is a target gene of spliced X-box binding protein 1 and strongly induced during plasma cell (PC) differentiation. OBJECTIVE: We identified a novel genetic defect and studied its pathologic mechanism in 11 patients from 2 unrelated families with PADs...
August 4, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28768817/cxcr4-specific-nanobodies-as-potential-therapeutics-for-whim-syndrome
#18
Raymond H de Wit, Raimond Heukers, Hendrik Brink, Angela Arsova, David Maussang, Pasquale Cutolo, Beatrijs Strubbe, Henry Vischer, Francoise Bachelerie, Martine J Smit
WHIM syndrome is a rare congenital immunodeficiency disease, named after its main clinical manifestations: Warts, Hypogammaglobulinemia, Infections and Myelokathexis. The disease is primarily caused by C-terminal truncation mutations of the chemokine receptor CXCR4. Consequently, these CXCR4-WHIM mutants have a gain of function in response to its ligand CXCL12, which results in abnormal leukocyte migration and thereby immune system dysfunctions. Treatment of WHIM patients currently consists of symptom relief, leading to unsatisfactory clinical responses...
August 2, 2017: Journal of Pharmacology and Experimental Therapeutics
https://www.readbyqxmd.com/read/28767614/primary-intestinal-lymphangiectasia-in-an-elderly-female-patient-a-case-report-on-a-rare-cause-of-secondary-immunodeficiency
#19
Xaver Huber, Lukas Degen, Simone Muenst, Marten Trendelenburg
Protein loss via the gut can be caused by a number of gastrointestinal disorders, among which intestinal lymphangiectasia has been described to not only lead to a loss of proteins but also to a loss of lymphocytes, resembling secondary immunodeficiency. We are reporting on a 75-year-old female patient who came to our hospital because of a minor stroke. She had no history of serious infections. During the diagnostic work-up, we detected an apparent immunodeficiency syndrome associated with primary intestinal lymphangiectasia...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28755066/two-sides-of-the-same-coin-pediatric-onset-and-adult-onset-common-variable-immune-deficiency
#20
Lauren A Sanchez, Solrun Melkorka Maggadottir, Matthew S Pantell, Patricia Lugar, Charlotte Cunningham Rundles, Kathleen E Sullivan
PURPOSE: Common variable immunodeficiency (CVID) is a complex, heterogeneous immunodeficiency characterized by hypogammaglobulinemia, recurrent infections, and poor antibody response to vaccination. While antibiotics and immunoglobulin prophylaxis have significantly reduced infectious complications, non-infectious complications of autoimmunity, inflammatory lung disease, enteropathy, and malignancy remain of great concern. Previous studies have suggested that CVID patients diagnosed in childhood are more severely affected by these complications than adults diagnosed later in life...
July 28, 2017: Journal of Clinical Immunology
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