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Risk factors of partial IgA deficiency among low serum IgA patients: a retrospective observational study.

IntroductionPartial IgA deficiency (pIgAD), including selective IgA deficiency, is one of the most common types of immunodeficiency. Early detection is crucial to prevent complications, such as recurrent infections and anaphylactic reactions to blood derivatives.Material and methodsUseful screening methods have not yet been established. We conducted a single-center retrospective observational study, with low serum IgA patients to clarify the risk factors of pIgAD among patients with low serum levels of IgA. All patients with low serum IgA levels treated in our outpatient clinic from April 2010

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… In addition, some patients develop common variable immunodeficiency (CVID) [ 26 , 27 ], and suffer from anaphylactic reactions to blood derivatives [ 28 ]. … Selective IgA deficiency (SIgAD) and common variable immunodeficiency (CVID). Clin Exp Immunol 120: 225-231. 13. Castigli E, Wilson SA, Garibyan L, et al. (2005): TACI is mutant in common variable immunodeficiency and IgA deficiency. Nat Genet 37: 829-834. 14. …

Subcutaneous immunoglobulins replacement therapy in secondary antibody deficiencies: Real life evidence as compared to primary antibody deficiencies.

Secondary antibody deficiencies (SAD) may require immunoglobulin replacement therapy (IgRT). While the intravenous route (IVIG) is broadly considered effective in SAD, the use of subcutaneous immunoglobulins (SCIG) is mainly adopted from the experience in primary antibody deficiencies (PAD), where SCIG have been shown to perform as effective as IVIG. However, evidence-based data on SCIG administration in SAD patients are still insufficient. Herein we retrospectively evaluated the efficacy and safety profile of SCIG treatment in 131 SAD patients as compared to a group of 102 PAD patients. We fo

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… Variable Immunodeficiency (CVID) was the diagnosis in 75/102 patients (of which 54/75 and 21/75 showed combined IgG, IgA and IgM or IgG and IgA reduction, respectively), 5/102 had isolated IgG subclass deficiency, 2/102 had IgA with IgG subclass deficiency, 1 patient had specific deficit of anti-pneumococcal … Infection outcomes in patients with common variable immunodeficiency disorders: relationship to immunoglobulin therapy over 22 years. J Allergy Clin Immunol. 2010. June;125(6):1354–1360.e4. 10.1016/j.jaci.2010.02.040 36. Windegger TM, Nghiem S, Nguyen K-H, Fung Y-L, Scuffham PA. …

Secondary antibody deficiencies in the modern era: emerging trends, diagnostic pitfalls, and advances in personalised management.

Secondary antibody deficiencies (SADs) are a significant but frequently under-recognised group of acquired immunodeficiencies. They may arise in various clinical settings, including haematological malignancies, immunosuppressive therapies, and protein-losing conditions. SADs are associated with an increased risk of recurrent and severe infections, hospitalisation, and impaired quality of life. Despite this, diagnostic and treatment pathways remain inconsistent across healthcare settings and regions. Recent advances in the use of structured clinical data, including electronic health records and

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… Although it shares some features with common variable immunodeficiency (CVID), it typically occurs later in life and has a distinct immunological profile ( 18 ). … Large-scale deep learning analysis to identify adult patients at risk for combined and common variable immunodeficiencies . Commun Med . ( 2023 ) 3 . doi: 10.1038/s43856-023-00412-8 , PMID: 38123736 PMC10733406 41 Johnson R Stephens AV Mester R Knyazev S LA K MK F . …

2018 Ежегодное совещание СНГ: Североамериканская конференция по вопросам неадекватности и регулирования.

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… Objectives: We describe a 15-year-old male patient with novel heterozygous mutation of EP300 gene; his first manifestations were initially characterized by infections, cytopenia and hypogammaglobulinemia suggesting a Common Variable Immunodeficiency (CVID), but later on, persisting lymphopenia was suggestive … Differential diagnosis included common variable immunodeficiency (CVID) or autoimmune lymphoproliferative syndrome and therefore a broad search for causative genetic defect was initiated. The parents are first cousins of Middle-Eastern origin suggesting an autosomal recessive inheritance. …

Health status and infections in patients with symptomatic primary and secondary immunoglobulin G (IgG) deficiencies receiving intravenous IgG replacement.

BackgroundThe effects of intravenous immunoglobulin G replacement on perceived health and infection susceptibility of patients suffering from immunoglobulin G (IgG) deficiencies should be evaluated in a prospective analysis.MethodsPatients with symptomatic primary or secondary IgG deficiencies were interviewed prior to the first IgG infusion (t0) and over the course of their treatment (t1 - t6). The respondents rated their current health using a 100 point scale (EQ-5D-5L), ranging from 0 ('worst imaginable health') to 100 ('best imaginable health'). The patients also provided information on th

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… Hajjar J Kutac C Rider NL Seeborg FO Scalchunes C Orange J Fatigue and the wear-off effect in adult patients with common variable immunodeficiency Clin Exp Immunol 2018 194 3 327 338 10.1111/cei.13210 30168848 PMC6231022 17. …

Access and use of immunoglobulins in secondary supportive cancer care: A systematic literature review.

BackgroundImmunoglobulin replacement therapy (IgRT) benefits patients with primary immuno deficiency (PID) originating from the innate or polygenic defects in the immune system. However, evidence supporting their therapeutic role is not as explicit in secondary immuno deficiency (SID) resulting from the treatment of haematological malignancies.ObjectivesThis study aimed to (1) create a dataset of relevant research papers, which explore the use of IgRT in SID for analysis, (2) assess the risk of bias within this dataset and (3) study the characteristics of these papers.DesignThis systematic rev

Infections, autoimmunity and immunodeficiencies are the leading etiologies of non-cystic fibrosis bronchiectasis in adults from the southwest of Colombia.

INTRODUCTION: Non-cystic fibrosis bronchiectasis is a complex medical condition with multiple etiologies, characterized by chronic productive cough and radiologic evidence of airway lumen dilation and wall thickening. Associated exacerbations and declining lung function contribute to increasing disability and mortality. There are no data about the prevalence of non-cystic fibrosis bronchiectasis etiologies in the Colombian population. OBJECTIVE: To investigate non-cystic fibrosis bronchiectasis etiology and clinical characteristics in adults evaluated in the southwest of Colombia. MATERIALS

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… After immunoglobulin evaluation (IgG, IgA, IgM, and IgE) in all participants, four patients were diagnosed with de novo predominantly antibody deficiencies: Two with common variable immunodeficiency and two with selective IgA deficiency. …

British Society for Immunology and United Kingdom Primary Immunodeficiency Network (UKPIN) consensus guideline for the management of immunoglobulin replacement therapy.

Currently, there is no guideline to support the use of immunoglobulin replacement therapy (IgRT) in primary and secondary immunodeficiency disorders in UK. The UK Primary Immunodeficiency Network (UK-PIN) and the British Society of Immunology (BSI) joined forces to address this need. Given the paucity of evidence, a modified Delphi approach was used covering statements for the initiation, monitoring, discontinuation of IgRT as well as home therapy programme. A group of six consultant immunologists and three nurse specialists created the statements, reviewed responses and feedback and agreed on

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… Relationship of the dose of intravenous gammaglobulin to the prevention of infections in adults with common variable immunodeficiency . Inflammation 1996 , 20 , 353 – 9 . 8872499 10.1007/BF01486738 8. White WB , Desbonnet CR , Ballow M. … Bioavailability of gamma-globulin after subcutaneous infusions in patients with common variable immunodeficiency . J Clin Immunol 1994 , 14 , 90 – 7 . 7515071 10.1007/BF01541341 20. Wasserman RL , Irani A-M , Tracy J , et al. …

Access and use of immunoglobulins in supportive cancer care: A thematic analysis of a systematic review data set.

BackgroundSecondary immunodeficiency (SID) disorders are known to occur in patients with haematological malignancies (HM) due to immunosuppressive treatments. Recurring infections causing subsequent morbidity and mortality commonly occur in this patient cohort. Immunoglobulin replacement therapy (IgRT) benefits patients with primary antibody deficiencies. However, evidence supporting their therapeutic role is not as explicit in SID-associated antibody deficiencies, which raises the questions regarding its use in SID and the knock-on effects of this use on its access and availability more gener

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… variable immunodeficiency, among other diseases. … The patient had presented initially with a case of refractory urticaria which was deemed to be a rare symptom of her HGG that is often only seen in common variable immunodeficiency. …

Ataxia-Telangiectasia and Associated Bronchiectasis: Case Report and Literature Review.

Ataxia-telangiectasia is a rare, autosomal recessive primary immunodeficiency caused by mutations in the ATM gene on chromosome 11, which encodes a serine-threonine kinase essential for the recognition and repair of DNA double-strand breaks. The disease is characterized by progressive neurological impairment, immunological dysfunction, and an increased susceptibility to recurrent infections and malignancies. Pulmonary involvement represents a major source of morbidity and frequently arises from chronic infections, aspiration, and impaired airway clearance, ultimately leading to the development

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… Among primary immunodeficiencies causing pediatric bronchiectasis, the most common are immunoglobulin-related defects (70–75% of cases), including X-linked agammaglobulinemia, common variable immunodeficiency, IgG subclass deficiencies, selective IgA deficiency, hyper-IgE (Job) syndrome, hyper-IgM syndrome … Non-Hodgkin lymphoma in common variable immunodeficiency. Am. J. Hematol. 1991;37:69–74. doi: 10.1002/ajh.2830370202. 33. McShane P., Patel N., MacMahon H., King C.S., Mosenifar Z. Bronchiectasis in hematologic malignancy. Chest. 2011;140:458A. doi: 10.1378/chest.1115438. 34. Zo S., Moon J. …

Calculated globulin (CG) as a screening test for antibody deficiency.

Calculated globulin (total protein - albumin) is usually tested as part of a liver function test profile in both primary and secondary care and determines the serum globulin concentration, of which immunoglobulins are a major component. The main use hitherto of calculated globulin is to detect paraproteins when the level is high. This study investigated the potential to use low levels of calculated globulin to detect antibody deficiency. Serum samples with calculated globulin cut-off < 18 g/l based on results of a pilot study were collected from nine hospitals in Wales over a 12-month period.

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… Table 1 Causes of a high or low calculated globulin EBV = Epstein–Barr virus; HBC = hepatitis B core antigen; CVID = common variable immunodeficiency; TB = tuberculosis; ITU = intensive care unit. … The variable in common variable immunodeficiency: a disease of complex phenotypes. J Allergy Clin Immunol Pract. 2013;1:545–556. doi: 10.1016/j.jaip.2013.09.015. 2. Cunningham-Rundles C. How I treat common variable immune deficiency. …

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… variable immunodeficiency (CVID) Specific cell-mediated immunodeficiencies Severe combined immunodeficiency (SCID) Adenosine deaminase deficiency (ADA) SCID T − –B − (absence of T and B lymphocytes) SCID T − –B + (absence of T lymphocytes and NK cells) Protein kinase Jak-3 deficiency Protein kinase … Table 3 Overview of disorders that can be effectively treated using intravenous preparations of normal human immunoglobulin Very likely effective Primary immunodeficiencies X-linked agammaglobulinaemia Common variable immunodeficiency Selective IgG deficiency Severe combined immunodeficiency (SCID) Secondary …

Burden of Seasonal Human Coronavirus Infections in Hematopoietic Cell Transplant Recipients.

BackgroundCommunity respiratory viruses, such as seasonal human coronavirus (HCoV), commonly infect hematopoietic cell transplant (HCT) recipients. Recognizing the risk factors and outcomes of HCoV infections in HCT recipients is essential for the future development of potentially lifesaving therapeutics.MethodsWe performed a retrospective review of all HCoV-infected HCT recipients from September 1, 2015 to August 31, 2017, at our institution. Patients were classified with upper respiratory tract infection (URI) or lower respiratory infection (LRI) based on predefined definitions for respirato

Antibody deficiencies with normal IgG in adults with Non-cystic fibrosis bronchiectasis or recurrent pneumonia: Cross-sectional study.

BACKGROUND: Inborn errors of immunity, mainly Predominantly Antibody deficiencies with normal IgG levels are unrecognized in adults with lung diseases such as bronchiectasis or recurrent pneumonia. OBJECTIVE: To determine IgM, IgA, IgG2 subclass deficiencies, and Specific antibody deficiency (anti-pneumococcal polysaccharide antibodies) in adults with non-cystic fibrosis bronchiectasis or recurrent pneumonia. METHODS: Cross-sectional study. Consecutive patients with non-cystic fibrosis bronchiectasis or recurrent pneumonia were recruited in Cali, Colombia. IgG, IgA, IgM, and IgE, IgG2subclas

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… In our study, Common Variable Immunodeficiency frequency is slightly higher than reported in other series with recurrent upper/lower respiratory infections 14 , 21 . … Disseminated Bronchiectasis in an adult with Common Variable Immunodeficiency. Colomb Med (Cali) 2015;46(1):47–50. doi: 10.25100/cm.v46i1.1738. 12. …

Chronic Lymphocytic Leukemia-Induced Humoral Immunosuppression: A Systematic Review.

Secondary immunodeficiency is observed in all patients with chronic lymphocytic leukemia (CLL) in varying degrees. The aim of the study was to review the available literature data on patients with CLL, with particular regard to the pathogenesis of the disease and the impact of humoral immunity deficiency on the clinical and therapeutic approach. A systematic literature review was carried out by two independent authors who searched PubMed databases for studies published up to January 2020. Additionally, Google Scholar was used to evaluate search results and support manual research. The search r

Agents contributing to secondary immunodeficiency development in patients with multiple myeloma, chronic lymphocytic leukemia and non-Hodgkin lymphoma: A systematic literature review.

IntroductionPatients with hematological malignancies (HMs), like chronic lymphocytic leukemia (CLL), multiple myeloma (MM), and non-Hodgkin lymphoma (NHL), have a high risk of secondary immunodeficiency (SID), SID-related infections, and mortality. Here, we report the results of a systematic literature review on the potential association of various cancer regimens with infection rates, neutropenia, lymphocytopenia, or hypogammaglobulinemia, indicative of SID.MethodsA systematic literature search was performed in 03/2022 using PubMed to search for clinical trials that mentioned in the title and

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… Malignancy in common variable immunodeficiency: A systematic review and meta-analysis . Expert Rev Clin Immunol ( 2019 ) 15 ( 10 ): 1105–13 . doi: 10.1080/1744666X.2019.1658523 31452405 19 Guevara-Hoyer K Fuentes-Antrás J de la Fuente-Muñoz E Fernández-Arquero M Solano F Pérez-Segura P . … Genomic crossroads between non-hodgkin’s lymphoma and common variable immunodeficiency . Front Immunol ( 2022 ) 13 : 937872 . doi: 10.3389/fimmu.2022.937872 35990641 PMC9390007 20 Marshall JS Warrington R Watson W Kim HL . An introduction to immunology and immunopathology . …

Cancer incidence in immunocompromised patients: a single-center cohort study.

BackgroundDiminished immune defense plays an important role in cancer development. Cancer risk in immunocompromised patients may differ. Identifying individuals with elevated cancer risk can inform strategies for routine cancer screening. This study aimed to understand and compare cancer incidence and risk in three patient groups: recipients of solid organ transplant (SOT) or hematopoietic stem cell transplant (HSCT); diagnosis of primary or secondary immunodeficiency disorder (PID/SID); and recipients of tumor necrosis factor inhibitor (TNF-i) therapy.MethodsThis retrospective cohort study us

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… In addition to the specific PID disorders that have shown to increase the risk of cancer (common variable immunodeficiency disorder, severe combined immunodeficiency disorder, Wiskott-Aldrich syndrome, hypogammaglobulinemia) [ 13 , 25 ], the PID/SID cohort in this study also captured a variety of additional …

Clinicopathological features and prognostic factors of AIDS-related lymphoma: a retrospective single-center study in China.

AIDS-related lymphoma (ARL) is a leading cause of mortality among people living with HIV (PLWH), characterized by distinct clinicopathological features and a generally poor prognosis. However, comprehensive studies on ARL remain limited. This study aimed to evaluate the clinicopathological characteristics, immune status, and Epstein-Barr virus (EBV)/HIV viral loads in PLWH diagnosed with lymphoma, and to assess their prognostic significance. A retrospective analysis was conducted on 130 ARL cases diagnosed between 2017 and 2024. The cohort included 56 Burkitt lymphoma (BL), 51 diffuse large B-

Clinical Features and Outcomes of Pediatric and Adult Patients Hospitalized for Coronavirus Disease 2019: A Comparison Across Age Strata.

BackgroundCoronavirus disease 2019 (COVID-19) continues to cause hospitalizations and severe disease in children and adults.MethodsThis study compared the risk factors, symptoms, and outcomes of children and adults hospitalized for COVID-19 from March 2020 to May 2023 across age strata at 5 US sites participating in the Predicting Viral-Associated Inflammatory Disease Severity in Children with Laboratory Diagnostics and Artificial Intelligence consortium. Eligible patients had an upper respiratory swab that tested positive for severe acute respiratory syndrome coronavirus 2 by nucleic acid amp

Novel intravenous human IVIG in patients with secondary immunodeficiency: Interim analysis of a multicenter, prospective, non-interventional study.

OBJECTIVE: To evaluate the effectiveness, safety, and tolerability of Yimmug, a novel, highly purified, 10% human plasma-based intravenous immunoglobulin (IVIG) preparation, in patients with secondary immunodeficiency (SID) under real-world conditions. MATERIALS AND METHODS: This interim analysis is based on data from a multicenter, prospective, non-interventional study conducted with out-patients in Germany. Effectiveness was assessed by changes in (i) serum IgG levels, (ii) severe infection rates, (iii) clinical symptoms, and (iv) patient-reported quality of life (QoL) at three treatment in