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Marc D Cohen, Edward Keystone
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. Rituximab was efficacious in clinical trials of patients with RA, including those who are methotrexate naïve, those with an incomplete response to methotrexate, and those with an incomplete response to tumor necrosis factor inhibitors...
December 2015: Rheumatol Ther
Murat Sütçü, Hacer Aktürk, Nuran Salman, Deniz Özçeker, Sezen Gülümser-Şişko, Manolya Acar, Ayper Somer
This study evaluates the clinical/immunological features and outcomes of 91 patients with the diagnosis of transient hypogammaglobulinemia of infancy(THI). Mean age at diagnosis was 8.4±5.2 months. IgG levels normalized at 30.6±11.88 months. Sixty three patients (69.3%) resolved in their first 3 years of life and 28 patients (30.7%) thereafter. In the univariate analysis, presence of atopy, occurrence of recurrent infections (>6/year) and hospitalization, initial low IgA and IgM levels were found to be associated with the late recovery...
November 2015: Turkish Journal of Pediatrics
Erez Rechavi, Atar Lev, Eran Eyal, Ortal Barel, Nitzan Kol, Sarit Farage Barhom, Ben Pode-Shakked, Yair Anikster, Raz Somech, Amos J Simon
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. We sought to further investigate the immune defect in an ICF patient with a novel missense mutation in DNMT3B and a severe phenotype. METHODS: Patient lymphocytes were examined for subset counts, immunoglobulin levels, T and B cell de novo production (via excision circles) and receptor repertoire diversity...
October 12, 2016: Journal of Clinical Immunology
Masato Miyoshi, Sei Kakinuma, Yoko Tanabe, Koji Ishii, Tian-Cheng Li, Takaji Wakita, Yukio Tsuura, Hideki Watanabe, Yasuhiro Asahina, Mamoru Watanabe, Takaaki Ikeda
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. This case indicates that HEV infection should be ruled out during liver dysfunction in immunosuppressed or immunocompromised patients and suggests that an alternative therapeutic strategy for such patients will be needed...
2016: Internal Medicine
Shwetal Lalan, Hongying Dai, Bradley A Warady
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. Data collected from the start of CPD up to 2 years post-dialysis initiation included patient demographics, dialysis characteristics, serum immunoglobulin (IgG) levels, IVIG administration history, infectious complications and outcomes...
October 7, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Clairelyne Dupin, Sylvain Marchand-Adam, Olivier Favelle, Romain Costes, Philippe Gatault, Philippe Diot, Leslie Grammatico-Guillon, Laurent Guilleminault
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. Asthma patients with a serum immunoglobulin (Ig) quantitative analysis were included and classified into two groups depending on their serum IgG concentration: presence or absence of HGG. Clinical, biological, functional, and radiologic characteristics were compared in univariate and multivariate analysis, using a logistic regression model...
October 6, 2016: Journal of Clinical Immunology
Eva Hlavackova, Martin Liska, Hana Jicinska, Jiri Navratil, Jiri Litzman
The Fontan operation or total cavopulmonal connection (TCPC) is a palliative surgical correction of rare and complex inborn cardiac malformations that are characterized by univentricular circulation. Protein-losing enteropathy (PLE) develops in 4-13% of patients after the Fontan procedure. Fontan-related PLE leads to secondary combined immunodeficiency marked by hypogammaglobulinemia and predominantly CD4+ lymphocytopenia. Here, we present 3 case reports of patients with secondary immunodeficiency after the Fontan operation...
September 30, 2016: International Archives of Allergy and Immunology
E Sarmiento, P Diez, M Arraya, M Jaramillo, L Calahorra, J Fernandez-Yañez, J Palomo, I Sousa, J Hortal, J Barrios, R Alonso, P Muñoz, J Navarro, J Vicario, E Fernandez-Cruz, J Carbone
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...
September 17, 2016: Transplant Infectious Disease: An Official Journal of the Transplantation Society
Delfien J A Bogaert, Marieke De Bruyne, Veronique Debacker, Pauline Depuydt, Katleen De Preter, Carolien Bonroy, Jan Philippe', Victoria Bordon, Bart N Lambrecht, Tessa Kerre, Andrea Cerutti, Karim Y Vermaelen, Filomeen Haerynck, Melissa Dullaers
The etiology of primary antibody deficiencies is largely unknown. Beside rare monogenic forms, the majority of cases seem to have a more complex genetic basis. Whereas common variable immunodeficiency has been investigated in-depth, only few reports exist on milder primary antibody deficiencies like idiopathic primary hypogammaglobulinemia and IgG subclass deficiency. We performed flow cytometric immunophenotyping in 33 common variable immunodeficiency, 23 idiopathic primary hypogammaglobulinemia and 21 IgG subclass deficiency patients, and in 47 asymptomatic first-degree family members of patients and 101 unrelated healthy controls...
September 15, 2016: Haematologica
Sotirios Sachanas, Gerassimos A Pangalis, Petros Karouzakis, Efstathios Koulieris, Maria Moschogiannis, Christina Kalpadakis, Xanthi Yiakoumis, Dimitra Rontogianni
Malakoplakia, a rare granulomatous disease of infectious etiology, is commonly observed in immunocompromised patients. Chronic lymphocytic leukemia (CLL) is characterized by profound immune dysregulation resulting in significant infection-related morbidity and mortality, and several drugs used in CLL treatment have a severe immunosuppressive effect. Ibrutinib, has become a new standard-of-care in patients with CLL, especially for those harboring unfavorable genetic characteristics such as 17 p deletion, with however, unknown long-term immunological consequences...
September 2016: Anticancer Research
Seyhan Kutluğ, Gönül Ogur, Aysegül Yilmaz, Peter E Thijssen, Ummet Abur, Alisan Yildiran
ICF syndrome is a primary immunodeficiency disease characterized by hypo- or agammaglobulinemia, centromeric instability mainly on chromosomes 1, 9, and 16 and facial anomalies. ICF syndrome presents with frequent respiratory tract infections in infancy. A 20-month-old female patient was referred to our clinic due to frequent lower respiratory tract infections. ICF syndrome was considered because of comorbidity of hypogammaglobulinemia, facial anomalies, and neuromotor growth retardation. Metaphase chromosome analysis revealed centromeric instability on chromosomes 1, 9, and 16 and through Sanger a previously unreported homozygous missense mutation (c...
September 8, 2016: American Journal of Medical Genetics. Part A
Surjit Singh, Amit Rawat, Deepti Suri, Anju Gupta, Ravinder Garg, Biman Saikia, Ranjana Walker Minz, Shobha Sehgal, Koon-Wing Chan, Yu Lung Lau, Chikako Kamae, Kenichi Honma, Noriko Nakagawa, Kohsuke Imai, Shigeaki Nonoyama, Koichi Oshima, Noriko Mitsuiki, Osamu Ohara
BACKGROUND: X-linked agammaglobulinemia (XLA) is an X-linked genetic defect in maturation of B lymphocytes that results in the absence of B lymphocytes in the peripheral blood and profound hypogammaglobulinemia. It is caused by a mutation in the BTK gene located on the X chromosome. There are no large series describing XLA from the developing world. OBJECTIVE: To analyze the clinical features, immunologic and genetic characteristics, and outcomes of 36 patients with XLA diagnosed and managed for a period of 2 decades...
October 2016: Annals of Allergy, Asthma & Immunology
Sarah C Sasson, Sarah Davies, Raymond Chan, Leo Davies, Roger Garsia
BACKGROUND: Patients with thymoma with immunodeficiency (TWI)/Good's syndrome characteristically have evidence of combined immunodeficiency including low or absent B-cells, hypogammaglobulinemia and defects in T-cell mediated immunity. These patients can present with common or opportunistic infections. CASE PRESENTATION: A 54-year-old female was diagnosed with cerebral toxoplasmosis. This occurred on a background of metastatic thymoma previously treated with chemotherapy and myasthenia gravis (MG) treated with mycophenolate mofetil, monthly intravenous immunoglobulin (IVIG) and pyridostigmine...
2016: BMC Infectious Diseases
Elif Gülsüm Ümit, Figen Kuloğlu, Ahmet Muzaffer Demir
BACKGROUND: Vancomycin resistant enterococci (VRE) are common pathogens of hospital-acquired infection. Long hospitalization periods, use of broad-spectrum antibiotics and immunosuppression are major risks for VRE colonization. AIMS: We aimed to evaluate patients' characteristics and factors which may contribute to VRE colonization. METHODS: Data of 66 patients with colonization and 112 patients without colonization who have been hospitalized in the Hematology clinic were collected...
August 16, 2016: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
Rémi Bertinchamp, Laurence Gérard, David Boutboul, Marion Malphettes, Claire Fieschi, Eric Oksenhendler
BACKGROUND: In 2014, the European Society for Immune Deficiencies (ESID) revised the common variable immunodeficiency (CVID) diagnosis criteria by incorporating new clinical and biological markers. The new definition appeared more restrictive but had not yet been evaluated in a large cohort of patients. OBJECTIVE: The objective of this study was to evaluate the impact of this new definition in a large cohort of patients with primary hypogammaglobulinemia. METHODS: Evaluation of 3 different CVID definitions (ESID/Pan-American Group for Immunodeficiency [PAGID] 1999, ESID 2014, DEFI 2015) in 521 patients included in the French DEFI study with a diagnosis of primary hypogammaglobulinemia...
August 10, 2016: Journal of Allergy and Clinical Immunology in Practice
W C Wu, W Wang, H M Song, M S Ma, X Y Tang, S Jian, M Q Zhang, J Xiao, Z Q Qiu, Y L Liu
OBJECTIVE: To summarize and report the clinical characteristics and laboratory results of a case and those reported in literature with MHC class Ⅱ deficiency. METHOD: The clinical features, laboratory results and gene mutation analysis of an infant with MHC class Ⅱ deficiency, who was diagnosed and treated in Peking Union Medical College Hospital since December 2013, were retrospectively analyzed."Major histocompatibility complex class Ⅱ deficiency"or"bare lymphocyte syndrome"were used as keywords in order to retrieve reports from CNKI (from its establishment to October 2015) and Wanfang Database (from its establishment to October 2015), PubMed Database (from its establishment to October 2015) was searched...
August 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Kenneth K Yu, Constantin A Dasanu
Alemtuzumab is FDA-approved for the treatment of chronic lymphocytic leukemia (CLL). Nonetheless, its use for this indication has fallen out of favor due to serious concerns for infectious complications and increased risks of second malignancies from the profound and lasting immunosuppression. We report here in a patient with a rapidly progressive metastatic Merkel cell carcinoma (MCC) who was previously treated with alemtuzumab and fludarabine for CLL. He developed profound lymphopenia and hypogammaglobulinemia...
June 2016: Connecticut Medicine
J-F Augusto, A-S Garnier, J Demiselle, V Langs, J Picquet, R Legall, C Sargentini, T Culty, C Poli, M Ammi, A Ducancelle, A Chevailler, A Duveau, J-F Subra, J Sayegh
BACKGROUND: Recent data have outlined a link between hypogammaglobulinemia (HGG) and infection risk and suggested that HGG correction may decrease post-transplant infections. METHODS: We analyzed the risk factors of HGG and the relationship between HGG and the risk of severe infection in a cohort of 318 kidney transplant recipients (KTR) who were transplanted between 2003 and 2013. Immunoglobulin (Ig) concentration was measured prospectively at day 15 (D15), month 6 (M6), month 12 (M12), and month 24 (M24) post transplant...
October 2016: Transplant Infectious Disease: An Official Journal of the Transplantation Society
David A Khan
OBJECTIVE: There has been a great expanse in the use of biological agents during the past decade. However, there are significant differences between biologics and typical pharmaceutical drugs. This review focuses on 3 separate types of adverse reactions to biologics, namely high cytokine reactions, hypersensitivity reactions, and secondary immunodeficiency. DATA SOURCES: A PubMed literature search restricted to the previous 10 years using combinations of search terms, including omalizumab, rituximab, TGN1412, biologic agent, anaphylaxis, hypogammaglobulinemia, desensitization, and cytokine storm, was performed...
August 2016: Annals of Allergy, Asthma & Immunology
Monica G Lawrence, Judith A Woodfolk, Alexander J Schuyler, Leland C Stillman, Martin D Chapman, Thomas A E Platts-Mills
We present results from clinical studies on plasma infusion done in the late 1970s in patients with hypogammaglobulinemia in which we documented the short half-life of both total and allergen-specific IgE in serum. The development of specific allergic sensitization in the skin of those patients followed by the gradual decrease in sensitization over 50 days was also documented. The data are included here along with a discussion of the existing literature about the half-life of IgE in both the circulation and skin...
July 14, 2016: Journal of Allergy and Clinical Immunology
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