2,617 results match your criteria Immunoglobulin M Deficiency


Clinical features and immunoglobulin replacement therapy outcomes of adults with common variable immunodeficiency: a single centre experience.

Turk J Med Sci 2021 May 23. Epub 2021 May 23.

Background And Aim: Common variable immunodeficiency (CVID) characterized by defective immunoglobulin production is the most prevalent form of symptomatic primary immunodeficiency (PID) in adults. We aimed to reveal the clinical features of adults with CVID and to evaluate the effects of immunoglobulin replacement treatment (IRT) on hemato-immunological findings.

Materials And Methods: This study included 26 adult patients receiving IRT. Read More

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Diagnostic and Management Errors in an Immunodeficient Patient with Pneumococcal Pneumonia with Bacteraemia due to Incorrect Assessment of the Patient's Immune Status.

Eur J Case Rep Intern Med 2021 26;8(4):002557. Epub 2021 Apr 26.

Department of Diagnostic and Generalist Medicine, Dokkyo Medical University Hospital, Mibu, Tochigi, Japan.

A 79-year-old woman presented with fever and pleural chest pain. Based on the assessment of mild community pneumonia in an immunocompetent patient, outpatient follow-up was planned. However, the patient was admitted several hours later with a diagnosis of pneumococcal pneumonia with bacteraemia. Read More

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Dermoscopic Features of Giant Molluscum Contagiosum in a Patient with Acquired Immunodeficiency Syndrome.

Acta Dermatovenerol Croat 2020 Dec;28(7):233-235

Martyna Sławińska MD, PhD, Department of Dermatology, Venereology and Allergology Medical University of Gdańsk, Smoluchowskiego 17 Street, 80-214 Gdańsk;

Giant molluscum contagiosum (MC) is a peculiar variant of the disease with the presence of multiple or single lesions larger than 5 mm. In contrast to typical molluscum contagiosum, dermoscopic features of giant lesions have been poorly described, and none of the reports included multiple giant lesions in an immunocompromised patient. We present a patient with acquired immunodeficiency syndrome diagnosed with multiple giant molluscum contagiosum along with the dermoscopic features of this entity. Read More

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December 2020

Beta-2-Glycoprotein-I Deficiency Could Precipitate an Antiphospholipid Syndrome-like Prothrombotic Situation in Patients With Coronavirus Disease 2019.

ACR Open Rheumatol 2021 Apr 19;3(4):267-276. Epub 2021 Mar 19.

Hospital 12 de Octubre, Healthcare Research Institute and Biomedical Research Centre Network for Epidemiology and Public Health, Madrid, Spain.

Objective: Patients with coronavirus disease 2019 (COVID-19) present coagulation abnormalities and thromboembolic events that resemble antiphospholipid syndrome (APS). This work has aimed to study the prevalence of APS-related antigens, antibodies, and immune complexes in patients with COVID-19 and their association with clinical events.

Methods: A prospective study was conducted on 474 adults with severe acute respiratory syndrome coronavirus 2 infection hospitalized in two Spanish university hospitals. Read More

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Selective immunoglobulin M deficiency complicated by systemic lupus erythematosus and antiphospholipid syndrome: a case report and review of literature.

CEN Case Rep 2021 Feb 22. Epub 2021 Feb 22.

Department of Nephrology, Teine Keijinkai Medical Center, Sapporo, Hokkaido, Japan.

Selective immunoglobulin M deficiency (SIgMD) is the isolated absence of serum immunoglobulin M (IgM) with normal levels of other serum immunoglobulins. SIgMD is associated with infections and autoimmune diseases. While there are few reports on SIgMD complicated by systemic lupus erythematosus (SLE), there are no reports on SIgMD complicated by SLE and antiphospholipid syndrome (APS); we present the first report of this kind. Read More

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February 2021

Progression of primary selective immunoglobulin M deficiency to common variable immunodeficiency.

Ann Allergy Asthma Immunol 2021 Jun 17;126(6):723-724. Epub 2021 Feb 17.

Division of Basic and Clinical Immunology, Department of Medicine, University of California, Irvine, Irvine, California. Electronic address:

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Selective immunoglobulin M deficiency in a patient with celiac disease and recurrent pneumonia.

Clin Case Rep 2021 Jan 18;9(1):158-163. Epub 2020 Dec 18.

Autoimmune Diseases Research Center Kashan University of Medical Sciences Kashan Iran.

SIgMD is a rare immune disorder that occurs in a primary or secondary condition. Patients with recurrent infectious, cancers, and autoimmune disorders should be investigated to determine SIgMD. Read More

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January 2021

Evaluation of SARS-CoV-2 Serological Testing in Patients with Multiple Myeloma and Other Hematologic Malignancies on Monoclonal Antibody Therapies.

Diagnostics (Basel) 2020 Nov 24;10(12). Epub 2020 Nov 24.

Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA.

Background: Patients with hematological malignancies (HM), including multiple myeloma (MM), frequently suffer from immune deficiency-associated infectious complications because of both the disease and the treatment. Alarming results from China and the UK confirm the vulnerability of HM patients to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection-driven coronavirus disease 2019 (COVID-19). Given that the immunoassay interference from the endogenous monoclonal immunoglobulin (M paraprotein) and treatment antibodies continually challenges the MM management, it is critical to evaluate the SARS-CoV-2 serology tests for suspected interference/cross-reactivity. Read More

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November 2020

SLy2-deficiency promotes B-1 cell immunity and triggers enhanced production of IgM and IgG antibodies against pneumococcal vaccine.

Immun Inflamm Dis 2020 12 24;8(4):736-752. Epub 2020 Oct 24.

Department of Pharmacology, Experimental Therapy and Toxicology, Institute of Experimental and Clinical Pharmacology and Pharmacogenomik and ICePhA, University of Tuebingen, Tuebingen, Germany.

Background: Despite the benefits of existing vaccines, Streptococcus pneumoniae is still responsible for the greatest proportion of respiratory tract infections around the globe, thereby substantially contributing to morbidity and mortality in humans. B-1 cells are key players of bacterial clearance during pneumococcal infection and even provide long-lasting immunity towards S. pneumoniae. Read More

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December 2020

Seropositivity rate and diagnostic accuracy of serological tests in 2019-nCoV cases: a pooled analysis of individual studies.

Authors:
C-C Guo J-Q Mi H Nie

Eur Rev Med Pharmacol Sci 2020 Oct;24(19):10208-10218

Department of Pathology, Department of Nuclear Medicine; The First Affiliated Hospital, and College of Clinical Medicine of Henan University of Science and Technology, Luoyang, China.

Objective: Currently, detection of SARS-CoV-2 RNA is standard in the diagnosis of COVID-19 (2019-nCoV). However, reliable and rapid serological diagnostic methods to screen SARS-CoV-2 infected patients, including those who do not have overt symptoms, are urgently needed. Most studies have described serological tests based on the detection of SARS-CoV-2-specific IgM and IgG. Read More

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October 2020

Neurolisteriosis in a previously asymptomatic patient with serum IgM deficiency: a case report.

BMC Neurol 2020 Aug 31;20(1):323. Epub 2020 Aug 31.

Department of Medical Biopathology, Eginition Hospital, National and Kapodistrian University of Athens, Medical School, Athens, Greece.

Background: Listeria monocytogenes is an opportunistic pathogen of the central nervous system commonly associated with impaired cell-mediated immunity. We hereby present a case of adult neurolisteriosis where the only immunological feature persistently present was serum IgM deficiency, suggesting that non-specific humoral immunity may also play a central role in the control of neuroinvasion by Listeria monocytogenes.

Case Presentation: A 62-year-old male who had never experienced severe infections presented with headache, nuchal rigidity and confusion. Read More

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Association of Epstein-Barr virus with regression after withdrawal of immunosuppressive drugs and subsequent progression of iatrogenic immunodeficiency-associated lymphoproliferative disorders in patients with autoimmune diseases.

Hematol Oncol 2020 Dec 21;38(5):799-807. Epub 2020 Aug 21.

Department of Surgical Pathology, Hokkaido University Hospital, Sapporo, Hokkaido, Japan.

Patients with autoimmune diseases (AIDs) may develop lymphoproliferative disorders (LPDs) during treatment with immunosuppressive agents (IS) such as methotrexate (MTX), biological agents, or tacrolimus. Some LPDs in patients with AIDs (AID-LPDs) regress after withdrawal of IS, and a high incidence of Epstein-Barr virus (EBV) positivity in such patients has been reported. To identify characteristics and factors predictive of the response to treatment and disease progression, we retrospectively analyzed clinical and histopathological data for 81 patients with AID-LPDs. Read More

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December 2020

IgM, IgA and IgG response to conjugate polysaccharides in children with recurrent respiratory infections.

Scand J Immunol 2021 Jan 27;93(1):e12955. Epub 2020 Aug 27.

Pediatric Immunopathology and Allergology Unit, Policlinico Tor Vergata, University of Rome Tor Vergata, Rome, Italy.

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January 2021

Evaluation of pulmonary findings in patients with humoral immunodeficiency.

Turk Pediatri Ars 2020 19;55(2):174-183. Epub 2020 Jun 19.

Division of Pediatric Allergy and Immunology, Department of Pediatrics, Uludağ University, Faculty of Medicine, Bursa, Turkey.

Aim: To determine the frequency of sinopulmonary infections, detect changes in the respiratory system, and measure functional capacity of the lungs in our patients with humoral immunodeficiency.

Material And Methods: Fifty-six patients with humoral immunodeficiency were enrolled in this study. The clinical, laboratory, and radiologic data, and pulmonary function tests of the subjects were evaluated from their file records, retrospectively. Read More

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Prevalence and pathogenicity of autoantibodies in patients with idiopathic CD4 lymphopenia.

J Clin Invest 2020 10;130(10):5326-5337

HIV Pathogenesis Section, Laboratory of Immunoregulation, and.

BACKGROUNDIdiopathic CD4 lymphopenia (ICL) is defined by persistently low CD4+ cell counts (<300 cells/μL) in the absence of a causal infection or immune deficiency and can manifest with opportunistic infections. Approximately 30% of ICL patients develop autoimmune disease. The prevalence and breadth of their autoantibodies, however, and their potential contribution to pathogenesis of ICL remain unclear. Read More

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October 2020

Flow-cytometry as an auxiliary in the diagnosis of primary humoral immunodeficiencies.

Gac Med Mex 2020 ;156(3):194-200

Instituto Politécnico Nacional, Center of Research and Advanced Studies, Department of Molecular Biomedicine, Mexico City, Mexico.

Background: Antibody deficiencies encompass a wide spectrum of pathologies and constitute approximately 50 % of primary immunodeficiencies; with cytometry, it is possible to evaluate the immune status rapidly, effectively and at low cost.

Objective: To assess, by means of flow cytometry, the cells of patients with three types of primary humoral immunodeficiencies.

Method: Using flow cytometry, blood samples from patients and healthy subjects were analyzed with different monoclonal antibodies. Read More

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SARS-CoV-2 lgM/lgG antibody detection confirms the infection after three negative nucleic acid detection.

J Cell Mol Med 2020 07 19;24(14):8262-8265. Epub 2020 May 19.

Department of Cardiology, Zhongshan Hospital, Shanghai Institute of Cardiovascular Diseases, Fudan University, Shanghai, China.

An ongoing outbreak of viral pneumonia was caused by a novel coronavirus in China in 2019. By March 19, over 200 thousand confirmed cases of SARS-CoV-2 infection and over 9000 deaths have been reported throughout the world. For this infectious disease, nucleic acid detection is still the gold standard for pathogenic detection. Read More

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Treatment with a Toll-like Receptor 7 ligand evokes protective immunity against atherosclerosis in hypercholesterolaemic mice.

J Intern Med 2020 09 14;288(3):321-334. Epub 2020 May 14.

Laboratory of Immunobiology, Cardiovascular Medicine Unit, Department of Medicine, Center for Molecular Medicine, Karolinska Institutet and Karolinska University Hospital, Stockholm, Sweden.

Background: The interplay between innate and adaptive immunity is central in life-threatening clinical complications of atherosclerosis such as myocardial infarction and stroke. The specific mechanisms involved and their protective versus detrimental effects in the disease process remain poorly understood. We have previously shown that higher levels of Toll-like receptor 7 (TLR7) expression in human atherosclerotic lesions are correlated with better patient outcome. Read More

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September 2020

New S19 Mutant to Improve Distinction Between Infected and Vaccinated Animals.

Iran J Biotechnol 2019 Sep 1;17(3):e2159. Epub 2019 Sep 1.

Department of Microbiology, Faculty of Veterinary Medicine, University of Tehran, Tehran, Iran.

Background: Using Strain 19 (S19) to control bovine brucellosis is restricted due to induce antibodies to the O-side chain of the smooth lipopolysaccharide (LPS) which may be difficult to differentiate vaccinated and infected animals. Furthermore, it is virulent for humans and can induce abortion to cattle.

Objectives: The aim of this study was to employ gene knockout S19 for the first time to eliminate diagnostic defects and obtain the attenuated mutant strain. Read More

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September 2019

The epidemiology and clinical features of selective immunoglobulin M deficiency: A single-center study in China.

J Clin Lab Anal 2020 Jul 10;34(7):e23289. Epub 2020 Mar 10.

Department of Clinical Laboratory, Zhejiang Provincial People's Hospital, Hangzhou Medical College, Hangzhou, China.

Background: Selective immunoglobulin M deficiency (SIgMD) is a rare primary immunodeficiency that is frequently reported in Western countries. However, large epidemiological and clinical studies of SIgMD in China are still lacking. Herein, we describe a cohort of SIgMD subjects in a large tertiary university hospital in China. Read More

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CMTM7 plays key roles in TLR-induced plasma cell differentiation and p38 activation in murine B-1 B cells.

Eur J Immunol 2020 06 18;50(6):809-821. Epub 2020 Feb 18.

Department of Immunology, School of Basic Medical Sciences, Peking University Health Science Center, NHC Key Laboratory of Medical Immunology (Peking University), Beijing, China.

Terminal differentiation of B cells into antibody-secreting cells is the foundation of humoral immune response. B-1 cells, which are different from B-2 cells, preferentially differentiate into plasma cells. CMTM7 is a MARVEL-domain-containing membrane protein predominantly expressed in B cells that plays an important role in B-1a cell development. Read More

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Genetic loss of NFAT2 (NFATc1) impairs B cell development of B1 and B2 B cells.

Cell Immunol 2020 03 28;349:104048. Epub 2020 Jan 28.

Dept. of Hematology, Oncology and Immunology, University Hospital Tübingen, Tübingen, Germany; Dept. of Hematology, Oncology and Immunology, Klinikum Region Hannover, KRH Klinikum Siloah, Hannover, Germany. Electronic address:

NFAT2 activity was shown to be of critical importance in B cell receptor signaling, development and proliferation; however its role in B cell development in the periphery is still not completely understood. We confirmed that NFAT2 deletion leads to impaired B1 B cell development, supported by our finding of limited B1 progenitors in the bone marrow and spleen of NFAT2 deficient mice. Moreover, we show for the first time that loss of NFAT2 increases immature B cells in particular transitional T2 and T3 as well as mature follicular B cells while marginal zone B cells are decreased. Read More

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Lack of Gut Secretory Immunoglobulin A in Memory B-Cell Dysfunction-Associated Disorders: A Possible Gut-Spleen Axis.

Front Immunol 2019 8;10:2937. Epub 2020 Jan 8.

Department of Molecular Medicine, Sapienza University, Rome, Italy.

B-1a B cells and gut secretory IgA (SIgA) are absent in asplenic mice. Human immunoglobulin M (IgM) memory B cells, which are functionally equivalent to mouse B-1a B cells, are reduced after splenectomy. To demonstrate whether IgM memory B cells are necessary for generating IgA-secreting plasma cells in the human gut. Read More

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November 2020

Post-rituximab immunoglobulin M (IgM) hypogammaglobulinemia.

Autoimmun Rev 2020 Mar 7;19(3):102466. Epub 2020 Jan 7.

Department of Dermatology, Tufts University School of Medicine, The Center for Blistering Diseases, Boston, MA, USA. Electronic address:

Rituximab is a B cell depleting monoclonal antibody that targets the B cell-specific cell surface antigen CD20 and is currently used to treat several autoimmune diseases. The elimination of mature CD20-positive B lymphocytes committed to differentiate into autoantibody-producing plasma cells is considered to be the major effect of rituximab, that makes it a beneficial biological agent in treating autoimmune diseases. Hypogammaglobulinemia has been reported after rituximab therapy in patients with lymphoma and rheumatoid arthritis. Read More

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Associations of Interleukin-5 With Plaque Development and Cardiovascular Events.

JACC Basic Transl Sci 2019 Dec 6;4(8):891-902. Epub 2019 Nov 6.

Department of Clinical Sciences Malmö, Lund University, Lund, Sweden.

Experimental studies have suggested an atheroprotective role of interleukin (IL)-5 through the stimulation of natural immunoglobulin M antibody expression. In the present study we show that there are no associations between baseline levels of IL-5 and risk for development of coronary events or stroke during a 15.7 ± 6. Read More

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December 2019

Dietary biotin deficiency decreased growth performance and impaired the immune function of the head kidney, spleen and skin in on-growing grass carp (Ctenopharyngodon idella).

Fish Shellfish Immunol 2020 Feb 17;97:216-234. Epub 2019 Dec 17.

Animal Nutrition Institute, Sichuan Agricultural University, Sichuan, Chengdu, 611130, China; Fish Nutrition and Safety Production University Key Laboratory of Sichuan Province, Sichuan Agricultural University, Sichuan, Chengdu, 611130, China; Key Laboratory for Animal Disease-Resistance Nutrition of China Ministry of Education, Sichuan Agricultural University, Sichuan, Chengdu, 611130, China. Electronic address:

The aim of this study was to investigate the effects of dietary biotin deficiency on the growth performance and immune function of the head kidney, spleen and skin in on-growing grass carp (Ctenopharyngodon idella). A total of 540 on-growing grass carp (117.11 ± 0. Read More

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February 2020

Predominantly Antibody-Deficient Patients With Non-infectious Complications Have Reduced Naive B, Treg, Th17, and Tfh17 Cells.

Front Immunol 2019 15;10:2593. Epub 2019 Nov 15.

Department of Immunology and Pathology, Central Clinical School, Monash University and The Alfred Hospital, Melbourne, VIC, Australia.

Patients with predominantly antibody deficiency (PAD) suffer from severe and recurrent infections that require lifelong immunoglobulin replacement and prophylactic antibiotic treatment. Disease incidence is estimated to be 1:25,000 worldwide, and up to 68% of patients develop non-infectious complications (NIC) including autoimmunity, which are difficult to treat, causing high morbidity, and early mortality. Currently, the etiology of NIC is unknown, and there are no diagnostic and prognostic markers to identify patients at risk. Read More

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November 2020

Rare TACI Mutation in a 3-Year-Old Boy With CVID Phenotype.

Front Pediatr 2019 15;7:418. Epub 2019 Oct 15.

Division of Pediatric Immunology and Rheumatology, Department of Pediatrics, Sapienza University of Rome, Rome, Italy.

Common variable immunodeficiency (CVID) is the most common and clinically relevant primary immunodeficiency (PID). Genetic basis of CVID remains largely unknown. However, in a minority of CVID patients, a number of distinct genetic defects affecting the normal processes of B cell maturation and differentiation into memory B cells have now been identified, resulting in markedly reduced serum levels of immunoglobulin G (IgG) and low immunoglobulin A (IgA) or immunoglobulin M (IgM), with impaired antibody responses, despite the presence of normal levels of B cells. Read More

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October 2019

Impairment of PD-L2 positive B1a cells enhances susceptibility to sepsis in RasGRP1-deficient mice.

Cell Immunol 2019 12 16;346:103993. Epub 2019 Oct 16.

Center for Immunobiology, Western Michigan University Homer Stryker M.D. School of Medicine, Kalamazoo, MI, USA; Department of Biomedical Sciences, Western Michigan University Homer Stryker M.D. School of Medicine, Kalamazoo, MI, USA.

RasGRP1 is a key molecule that mediates antigen-initiated signaling for activation of the RAS-MAPK pathway in lymphocytes. Patients with aberrant RasGRP1 expression experience lymphocyte dysfunction and are afflicted with recurrent microbial infections. Yet, the underlying mechanism that accounts for microbial infection remains unknown. Read More

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December 2019