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Hypogammaglobulinemia

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https://www.readbyqxmd.com/read/29755528/hypogammaglobulinemia-observed-one-year-after-rituximab-treatment-for-idiopathic-thrombocytopenic-purpura
#1
Bilal Ahmad Shoukat, Osama Ali, Dileep Kumar, Muhammad Bilal Gilani, Adeela Zahid, Shaheer Aslam Joiya, Maqsood Anwar Malik
We present the case of a 19-year-old female with severe hypogammaglobulinemia after having had treatment with rituximab for idiopathic thrombocytopenic purpura requiring intravenous immunoglobulins. She was admitted with the diagnosis of left-sided pneumonia with parapneumonic effusion. The patient was treated with piperacillin/tazobactam after having a poor response to co-amoxiclav. The patient had been tested for immunoglobulin levels, and the levels were very low. She has a history of ITP for which she received steroids...
2018: Case Reports in Medicine
https://www.readbyqxmd.com/read/29754764/monitoring-of-early-humoral-immunity-to-identify-lung-recipients-at-risk-for-development-of-serious-infections-a-multicenter-prospective-study
#2
Elizabeth Sarmiento, Jose Cifrian, Leticia Calahorra, Carles Bravo, Sonia Lopez, Rosalia Laporta, Piedad Ussetti, Amparo Sole, Carmen Morales, Alicia de Pablos, Maria Jaramillo, Ikram Ezzahouri, Sandra García, Joaquin Navarro, Marcos Lopez-Hoyos, Javier Carbone
BACKGROUND: Infection is still a leading cause of death during the first year after lung transplantation. We performed a multicenter study among teaching hospitals to assess monitoring of early humoral immunity as a means of identifying lung recipients at risk of serious infections. METHODS: We prospectively analyzed 82 adult lung recipients at 5 centers in Spain. Data were collected before transplantation and at 7 and 30 days after transplantation. Biomarkers included IgG, IgM, IgA, complement factors C3 and C4, titers of antibodies to pneumococcal polysaccharide antigens (IgG, IgA, IgM) and antibodies to cytomegalovirus (IgG), and serum B-cell activating factor (BAFF) levels...
April 6, 2018: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29752554/secondary-hypogammaglobulinemia-after-rituximab-for-neuromyelitis-optica-a-case-report
#3
Lara Farhat, Jasmeen Dara, Susan Duberstein, Aliva De
A 17-year-old male with history of neuromyelitis optica and seizures presented to the pulmonology clinic for evaluation of recurrent pneumonias. He had received rituximab for the past 6 years. Over the past 2 years, he experienced four episodes of pneumonia. In between these episodes, he would improve briefly but continued to have daily cough that was productive with yellow phlegm. He also had recurrent rhinitis and sinusitis despite multiple antibiotic courses. Review of chest X-rays revealed localized right middle lobe and right lower lobe infiltrates...
May 11, 2018: Drug Safety—Case Reports
https://www.readbyqxmd.com/read/29748530/bk-virus-a-cause-for-concern-in-thoracic-transplantation
#4
Markus J Barten, Andreas Zuckermann
Human BK polyomavirus (BKV) infection is poorly documented in heart and lung transplant patients. BK viruria and viremia have been estimated to affect 19% and 5% of heart transplant recipients, respectively. Data are limited, especially for lung transplantation, but the proportion of patients progressing from BK viruria to viremia or BKV-related nephropathy (BKVN) appears lower than in kidney transplantation. Nevertheless, a number of cases of BKVN have been reported in heart and lung transplant patients, typically with late diagnosis and generally poor outcomes...
May 11, 2018: Annals of Transplantation: Quarterly of the Polish Transplantation Society
https://www.readbyqxmd.com/read/29748028/pneumococcal-conjugate-vaccine-triggers-a-better-immune-response-than-pneumococcal-polysaccharide-vaccine-in-patients-with-chronic-lymphocytic-leukemia-a-randomized-study-by-the-swedish-cll-group
#5
Tobias Svensson, Magdalena Kättström, Ylva Hammarlund, Daniel Roth, P-O Andersson, Magnus Svensson, Ingmar Nilsson, Lars Rombo, Honar Cherif, Eva Kimby
AIM: To determine if patients with untreated chronic lymphocytic leukemia (CLL) benefit from vaccination with a 13-valent pneumococcal conjugated vaccine (PCV13), Prevenar13®, compared to a 23-valent pneumococcal polysaccharide vaccine (PPSV23), Pneumovax®, in terms of immune response. BACKGROUND: Streptococcus pneumoniae causes substantial morbidity in patients with CLL, a group known to respond poorly to polysaccharide vaccines. Comparative studies with conjugated vaccines are lacking...
May 7, 2018: Vaccine
https://www.readbyqxmd.com/read/29729943/phenotype-penetrance-and-treatment-of-133-ctla-4-insufficient-individuals
#6
Charlotte Schwab, Annemarie Gabrysch, Peter Olbrich, Virginia Patiño, Klaus Warnatz, Daniel Wolff, Akihiro Hoshino, Masao Kobayashi, Kohsuke Imai, Masatoshi Takagi, Ingunn Dybedal, Jamanda A Haddock, David Sansom, Jose M Lucena, Maximilian Seidl, Annette Schmitt-Gräff, Veronika Reiser, Florian Emmerich, Natalie Frede, Alla Bulashevska, Ulrich Salzer, Desirée Schubert, Seiichi Hayakawa, Satoshi Okada, Maria Kanariou, Zeynep Yesim Kucuk, Hugo Chapdelaine, Lenka Petruzelkova, Zdenek Sumnik, Anna Sediva, Mary Slatter, Peter D Arkwright, Andrew Cant, Hanns-Martin Lorenz, Thomas Giese, Vassilios Lougaris, Alessandro Plebani, Christina Price, Kathleen E Sullivan, Michel Moutschen, Jiri Litzman, Tomas Freiberger, Frank L van de Veerdonk, Mike Recher, Michael H Albert, Fabian Hauck, Suranjith Seneviratne, Jana Pachlopnik Schmid, Antonios Kolios, Gary Unglik, Christian Klemann, Carsten Speckmann, Stephan Ehl, Alan Leichtner, Richard Blumberg, Andre Franke, Scott Snapper, Sebastian Zeissig, Charlotte Cunningham-Rundles, Lisa Giulino-Roth, Olivier Elemento, Gregor Dückers, Tim Niehues, Eva Fronkova, Veronika Kanderová, Craig D Platt, Janet Chou, Talal Chatila, Raif Geha, Elizabeth McDermott, Su Bunn, Monika Kurzai, Ansgar Schulz, Laia Alsina, Ferran Casals, Angela Deyà-Martinez, Sophie Hambleton, Hirokazu Kanegane, Kjetil Taskén, Olaf Neth, Bodo Grimbacher
BACKGROUND: Cytotoxic-T-lymphocyte-antigen-4 (CTLA-4) is a negative immune regulator. Heterozygous CTLA4 germline mutations can cause a complex immune dysregulation syndrome in humans. OBJECTIVE: To characterize the penetrance, the clinical features and the best treatment options in 133 CTLA4 mutation carriers. METHODS: Genetics, clinical features, laboratory values, and outcome of treatment options were assessed in a worldwide cohort of CTLA4 mutation carriers...
May 3, 2018: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29715199/cxcr4-haploinsufficient-bone-marrow-transplantation-corrects-leukopenia-in-an-unconditioned-whim-syndrome-model
#7
Ji-Liang Gao, Erin Yim, Marie Siwicki, Alexander Yang, Qian Liu, Ari Azani, Albert Owusu-Ansah, David H McDermott, Philip M Murphy
For gene therapy of gain-of-function autosomal dominant diseases, either correcting or deleting the disease allele is potentially curative. To test whether there may be an advantage of one approach over the other for WHIM (warts, hypogammaglobulinemia, infections and myelokathexis) syndrome - a primary immunodeficiency disorder caused by gain-of-function autosomal dominant mutations in chemokine receptor CXCR4 - we performed competitive transplantation experiments using both lethally irradiated wild-type (Cxcr4+/+) and unconditioned WHIM (Cxcr4+/w) recipient mice...
May 1, 2018: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/29709555/genetic-screening-of-male-patients-with-primary-hypogammaglobulinemia-can-guide-diagnosis-and-clinical-management
#8
Nicolas Vince, Gaël Mouillot, Marion Malphettes, Sophie Limou, David Boutboul, Angélique Guignet, Véronique Bertrand, Philippe Pellet, Pierre-Antoine Gourraud, Patrice Debré, Eric Oksenhendler, Ioannis Théodorou, Claire Fieschi
The precise diagnosis of an immunodeficiency is sometimes difficult to assess, especially due to the large spectrum of phenotypic variation reported among patients. Common variable immunodeficiency disorders (CVID) do not have, for a large part, an identified genetic cause. The identification of a causal genetic mutation is important to confirm, or in some cases correct, the diagnosis. We screened >150 male patients with hypogammaglobulinemia for mutations in three genes involved in pediatric X-linked primary immunoglobulin deficiency: CD40LG, SH2D1A and BTK...
April 27, 2018: Human Immunology
https://www.readbyqxmd.com/read/29705243/a-kindred-with-mutant-ikaros-and-autoimmunity
#9
Erika Van Nieuwenhove, Josselyn E Garcia-Perez, Christine Helsen, Princess D Rodriguez, Pauline A van Schouwenburg, James Dooley, Susan Schlenner, Mirjam van der Burg, Els Verhoeyen, Rik Gijsbers, Seth Frietze, Hilde Schjerven, Isabelle Meyts, Frank Claessens, Stephanie Humblet-Baron, Carine Wouters, Adrian Liston
IKAROS (encoded by IKZF1) is an important hematopoietic transcription factor critical for early B cell differentiation, with major defects known to lead to low B cell numbers and hypogammaglobulinemia. More perplexing is the link between IKZF1 variants and autoimmunity, including polymorphisms associated with susceptibility to SLE, and recently, rare variants driving monogenic autoimmunity. We identified a novel p.L188V mutation in IKZF1 in the index patient and her father and found this mutation to lead to loss of DNA binding...
April 26, 2018: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29702054/prevalence-and-safety-of-intravenous-immunoglobulin-administration-during-maintenance-chemotherapy-in-children-with-acute-lymphoblastic-leukemia-in-first-complete-remission-a-health-maintenance-organization-perspective
#10
Patrick Van Winkle, Raoul Burchette, Raymond Kim, Rukmani Raghunathan, Naveen Qureshi
CONTEXT: Children with acute lymphoblastic leukemia (ALL) in first complete remission (CR1) experience hypogammaglobulinemia and are at risk of sepsis during maintenance chemotherapy. Intravenous immunoglobulin (IVIG) has been used to try to circumvent this risk, but no data exist regarding its safety and prevalence in a health maintenance organization. OBJECTIVE: To evaluate the prevalence and safety of IVIG in children with ALL in CR1 during maintenance chemotherapy...
April 9, 2018: Permanente Journal
https://www.readbyqxmd.com/read/29670776/-kit-d816v-positive-acute-mast-cell-leukemia-associated-with-normal-karyotype-acute-myeloid-leukemia
#11
Marta Lopes, Maria Dos Anjos Teixeira, Cláudia Casais, Vanessa Mesquita, Patrícia Seabra, Renata Cabral, José Palla-García, Catarina Lau, João Rodrigues, Maria Jara-Acevedo, Inês Freitas, Jose Ramón Vizcaíno, Jorge Coutinho, Luis Escribano, Alberto Orfao, Margarida Lima
Introduction: Mast cell (MC) leukemia (MCL) is extremely rare. We present a case of MCL diagnosed concomitantly with acute myeloblastic leukemia (AML). Case Report: A 41-year-old woman presented with asthenia, anorexia, fever, epigastralgia, and diarrhea. She had a maculopapular skin rash, hepatosplenomegaly, retroperitoneal adenopathies, pancytopenia, 6% blast cells (BC) and 20% MC in the peripheral blood, elevated lactate dehydrogenase, cholestasis, hypoalbuminemia, hypogammaglobulinemia, and increased serum tryptase (184  μ g/L)...
2018: Case Reports in Hematology
https://www.readbyqxmd.com/read/29661141/diagnostic-utility-of-monitoring-cytomegalovirus-specific-immunity-by-quantiferon-cytomegalovirus-assay-in-kidney-transplant-recipients
#12
Dominika Deborska-Materkowska, Agnieszka Perkowska-Ptasinska, Anna Sadowska, Jolanta Gozdowska, Michał Ciszek, Marta Serwanska-Swietek, Piotr Domagala, Dorota Miszewska-Szyszkowska, Elzbieta Sitarek, Agnieszka Jozwik, Artur Kwiatkowski, Magdalena Durlik
BACKGROUND: Despite universal prophylaxis, late cytomegalovirus (CMV) infection occurs in a high proportion of kidney transplant recipients. We evaluated whether a specific viral T-cell response allows for the better identification of recipients who are at high risk of CMV infection after prophylaxis withdrawal. METHODS: We conducted a prospective study in 19 pretransplant anti-CMV seronegative kidney graft recipients R- (18 from seropositive donors [D+] and one from a seronegative donor [D-]) and 67 seropositive recipients R(+) (59 from seropositive donors and eight from seronegative donors) who received antiviral prophylaxis with valganciclovir...
April 16, 2018: BMC Infectious Diseases
https://www.readbyqxmd.com/read/29659363/-late-diagnosis-of-whim-sydrome
#13
María V Paolini, Silvia Danielian, Emma Prieto, María Fernanda Tami, Matías M Oleastro, Diego S Fernández Romero
WHIM syndrome is a primary autosomal dominant immuno deficiency due to CXCR4 mutations characterized by mucocutaneous warts, hypogammaglobulinemia, recurrent bacterial infections and myelokathesis. Treatment consists in prophylactic antibiotics, immunoglobulin replacement and granulocyte or granulocyte/monocyte colony stimulating factors. We present the case of a 21 year old woman who showed leukopenia at 10 months of age and one year later multiple infections with hypogammaglobulinemia requiring intravenous immunoglobulin...
2018: Medicina
https://www.readbyqxmd.com/read/29644723/a-french-observational-study-describing-the-use-of-human-polyvalent-immunoglobulins-in-hematological-malignancy-associated-secondary-immunodeficiency
#14
Omar Benbrahim, Jean-François Viallard, Sylvain Choquet, Bruno Royer, Frédéric Bauduer, Olivier Decaux, Jean-Charles Crave, Yann Fardini, Pierre Clerson, Vincent Levy
OBJECTIVE: To describe the characteristics of patients suffering from secondary immunodeficiencies (SID) associated with hematological malignancies (HM), who started immunoglobulins replacement therapy (IgRT), physicians' expectations regarding IgRT and IgRT modalities. METHODS: Non-interventional, prospective French cross-sectional study. RESULTS: The analysis included 231 patients (66±12 years old) suffering from multiple myeloma (MM) (N=64), chronic lymphoid leukemia (CLL) (N=84), aggressive non-Hodgkin B-cell lymphoma (aNHL) (N=32), indolent NHL (N=39), acute leukemia (N=6), Hodgkin disease (N=6)...
April 12, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29627491/colorectal-malakoplakia-in-a-patient-with-hypogammaglobulinemia
#15
Henrik Thorlacius, Anna Jerkeman, Felicia-Elena Marginean, Ervin Toth
No abstract text is available yet for this article.
April 5, 2018: Gastrointestinal Endoscopy
https://www.readbyqxmd.com/read/29618830/evaluation-of-the-tlr-negative-regulatory-network-in-cvid-patients
#16
Roozbeh Sanaei, Nima Rezaei, Asghar Aghamohammadi, Ali-Akbar Delbandi, Shahram Teimourian, Reza Yazdani, Parsova Tavasolian, Fatemeh Kiaee, Nader Tajik
Common variable immunodeficiency (CVID), a clinically symptomatic primary immunodeficiency disease (PID), is characterized by hypogammaglobulinemia leading to recurrent infections and various complications. Recently, some defects in the signaling of TLRs have been identified in CVID patients which led us to investigate the expression of TLR4 and 9 negative regulatory molecules and their upregulation status following their activation. Using TaqMan real-time PCR, SOCS1, TNFAIP3, RFN216, and IRAK-M transcripts among peripheral blood mononuclear cells (PBMCs) were measured with/without TLR4 and 9 activations...
April 5, 2018: Genes and Immunity
https://www.readbyqxmd.com/read/29618687/-successful-cord-blood-transplantation-in-a-patient-with-adult-onset-common-variable-immunodeficiency
#17
Yuhei Harutani, Shinobu Tamura, Hiroshi Kobata, Takehiro Oiwa, Yusuke Yamashita, Toshiki Mushino, Hiroki Hosoi, Shogo Murata, Akinori Nishikawa, Kohsuke Imai, Tomohiro Morio, Takashi Sonoki
Common variable immunodeficiency (CVID) is the most frequently diagnosed congenital immunodeficiency and is characterized by dysfunctional antibody production. It often occurs at the age of ≥10 years. Here we reported a case of a 46-year-old man confirmed with adult-onset CVID. He was effectively treated with cord blood transplant (CBT). The patient was observed with repeated upper respiratory infection a few years back and was referred to our department owing to a marked decrease in neutrophil counts and progression of anemia...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29575308/neutropenia-hypogammaglobulinemia-and-pneumonia-a-case-of-whim-syndrome
#18
Francesco Saettini, Lucia D Notarangelo, Andrea Biondi, Sonia Bonanomi
No abstract text is available yet for this article.
March 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/29569510/consequences-of-b-cell-depleting-therapy-hypogammaglobulinemia-and-impaired-b-cell-reconstitution
#19
Keith A Sacco, Roshini S Abraham
Rituximab is a chimeric monoclonal antibody used to treat hematologic and autoimmune diseases by depleting CD20-expressing B cells. Patients may develop hypogammaglobulinemia following treatment, with some demonstrating failure of B-cell recovery. The true frequency of hypogammaglobulinemia and/or impaired B-cell reconstitution post rituximab is unknown due to the lack of prospective studies in different patient cohorts. The clinical significance remains controversial; some patients have recurrent infections while others are relatively asymptomatic...
March 23, 2018: Immunotherapy
https://www.readbyqxmd.com/read/29557626/a-case-report-incomplete-kawasaki-disease-in-a-hypogammaglobulin%C3%A3-mie-child
#20
Burçin Şanlidag, Ceyhun Balkan, Nerin Bahçeciler
Kawasaki Disease (KD) is a systemic autoimmune vasculitis that affects small and medium sized vessels. Main complication of Kawasaki Disease is coronary artery aneurism, which has higher risk in case of delayed diagnosis and treatment. Although, complete and incomplete KD cases in different types of immune deficiency diseases have been presented up to date, clinical course of KD in patients with hypogammaglobulinemia (HG) has not been reported. Herein, a case diagnosed as incomplete KD in a child with transient HG of infancy has been reported...
April 1, 2018: Archivos Argentinos de Pediatría
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