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Treatment patterns and burden of infection in patients with chronic lymphocytic leukemia and secondary immunodeficiency: a retrospective database study.

Patients with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) and secondary immunodeficiency disease (SID) are susceptible to severe, recurrent, or persistent infections. This retrospective cohort study assessed the burden of infection in patients with CLL/SLL with and without SID, and in immunoglobulin replacement therapy (IgRT)-treated and -untreated patients with CLL/SLL and SID. Anonymized data from the US Optum-Humedica database (Oct-1-2015-Mar-10-2020) were used. Eligible patients aged ≥ 18 years with a confirmed CLL/SLL diagnosis were assigned to cohorts (SID or no-SID

Precision medicine: The use of tailored therapy in primary immunodeficiencies.

Primary immunodeficiencies (PID) are rare, complex diseases that can be characterised by a spectrum of phenotypes, from increased susceptibility to infections to autoimmunity, allergy, auto-inflammatory diseases and predisposition to malignancy. With the introduction of genetic testing in these patients and wider use of next-Generation sequencing techniques, a higher number of pathogenic genetic variants and conditions have been identified, allowing the development of new, targeted treatments in PID. The concept of precision medicine, that aims to tailor the medical interventions to each patie

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… Common variable immunodeficiency The common variable immunodeficiency (CVID) is a heterogenous group of diseases that has been associated with specific genetic defects, that can present with low levels of IgG and IgA with or without low IgM levels, absent or lower antibody responses to vaccination and … APDS, Activated PI3K Delta Syndrome; CANDLE, Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated temperature; CAPS, cryopyrin-associated periodic syndrome; CGD, chronic granulomatous disease; CTLA-4, cytotoxic T lymphocyte antigen-4; CVID, common variable immunodeficiency; EMA, European …

Clinical Manifestations of Human Exposure to Fungi.

Biological particles, along with inorganic gaseous and particulate pollutants, constitute an ever-present component of the atmosphere and surfaces. Among these particles are fungal species colonizing almost all ecosystems, including the human body. Although inoffensive to most people, fungi can be responsible for several health problems, such as allergic fungal diseases and fungal infections. Worldwide fungal disease incidence is increasing, with new emerging fungal diseases appearing yearly. Reasons for this increase are the expansion of life expectancy, the number of immunocompromised patien

Case Report: Kidney Transplantation in a Patient With Acquired Agammaglobulinemia and SLE. Issues and Challenges.

Lupus nephritis in the context of Systemic Lupus Erythematosus (SLE) is characterized by an unpredicted course with remissions and flare-ups. Among others, it remains a significant cause of end-stage kidney disease (ESKD) in relatively young patients. Therapeutic regimens with newer immunosuppressive agents have been introduced in order to control SLE clinical manifestations more efficiently and limit organ damage induced by immune complex formation and sustained inflammation. Treatment is usually long-term, and the cumulative impact of immunosuppression is expressed through the increased freq

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… International consensus document (ICON): common variable immunodeficiency disorders. J Allergy Clin Immunol. (2016) 4:38–59. 10.1016/j.jaip.2015.07.025 9. Smith T, Cunningham-Rundles C. Primary B-cell immunodeficiencies. Human Immunol. (2019) 80:351–62. 10.1016/j.humimm.2018.10.015 10. … Acute rejection of a kidney transplant in a patient with common variable immunodeficiency: a case report. Transplant Proc. (2017) 49:380–5. 10.1016/j.transproceed.2016.12.014 30. Hogan MB, Wilson NW, Muchant DG. Renal transplantation in a patient with common variable immunodeficiency. …

Inborn Errors of Immunity and Cancer.

Inborn Errors of Immunity (IEI) are a heterogeneous group of disorders characterized by a defect in the function of at least one, and often more, components of the immune system. The aim of this narrative review is to discuss the epidemiology, the pathogenesis and the correct management of tumours in patients with IEI. PubMed was used to search for all of the studies published over the last 20 years using the keywords: "inborn errors of immunity" or "primary immunodeficiency" and "cancer" or "tumour" or "malignancy". Literature analysis showed that the overall risk for cancer in children with

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… Key stages of bone marrow B-cell maturation are defective in patients with common variable immunodeficiency disorders. J. Allergy Clin. Immunol. 2015;136:487–490. doi: 10.1016/j.jaci.2014.12.1943. 83. … Mutational Analysis of Human BAFF Receptor TNFRSF13C (BAFF-R) in Patients with Common Variable Immunodeficiency. J. Clin. Immunol. 2005;25:496–502. doi: 10.1007/s10875-005-5637-2. 90. Cunningham-Rundles C. The many faces of common variable immunodeficiency. …

Insights into Facilitated Subcutaneous Immunoglobulin Use in Patients with Secondary Immunodeficiency Diseases: A FIGARO Subgroup Analysis.

The Facilitated Immunoglobulin Administration Registry And Outcomes (FIGARO) Study was a European, multicenter, prospective, observational study conducted across Europe designed to provide insights on the clinical use and tolerability of facilitated subcutaneous immunoglobulin (fSCIG). Data herein are reported for the cohort of patients with secondary immunodeficiency (SID), with a subgroup analysis by age. The SID cohort included 31 patients: 1 pediatric, 15 adult, and 15 older adult patients. Over the 36-month observation period, the median monthly dose of fSCIG (30 g) and median monthly inf

A Single Center Study Investigating Clinical Outcomes of Testing for Multiple Myeloma and Immune Deficiency at Low Globulin Levels

Background Both primary (e.g. common variable immune deficiency, CVID) and secondary immune deficiency as well as multiple myeloma (MM) require medical intervention and treatment delay can exacerbate morbidity. This study investigated the potential importance of low levels of calculated globulin to detect immune deficiency and MM associated with immunoparesis (light chain, non-secretory MM). Methods One hundred and thirty-nine patient serum samples from community physicians and outpatient clinics for liver function tests with low calculated globulin (Results Approximately 47% of patients in th

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… The natural history of untreated primary hypogammaglobulinemia in adults: implications for the diagnosis and treatment of Common Variable Immunodeficiency Disorders (CVID). Front Immunol . 2019;10:1541. doi: 10.3389/fimmu.2019.01541 17. Holding S, Khan S, Sewell WA, Jolles S, Dore PC. … Genetic diagnosis using whole exome sequencing in common variable immunodeficiency. Front Immunol . 2016;7:220. doi: 10.3389/fimmu.2016.00220 19. Cunningham-Rundles C. Common variable immune deficiency: case studies. Blood . 2019;134(21):1787–1795. doi: 10.1182/blood.2019002062 20. …

Immediate adverse events to intravenous immunoglobulin in pediatric patients with inborn errors of immunity: A longitudinal study with a pre-infusion protocol.

INTRODUCTION: Immunoglobulin represents the main therapy for patients with inborn errors of immunity (IEI) and it is a safe procedure, but adverse events (AEs) can occur with variable frequencies. OBJECTIVE: To evaluate the frequency of immediate AEs to intravenous immunoglobulin (IVIG) regular therapy in a pediatric cohort with IEI after a pre-IVIG infusion protocol. METHODS: This was a longitudinal study from 2011 to 2019 at a tertiary pediatric hospital in Brazil. RESULTS: A total of 1736 infusions were studied in 70 patients with IEI, 46 (65.7%) of whom were males and whose median age w

The Use of Bone Marrow Transplantation (BMT) or Hematopoietic Stem Cell Transplantation (HSCT) in Pediatric Patients Diagnosed With Ataxia-Telangiectasia: A Systematic Review.

Ataxia-telangiectasia (A-T) is a rare neurological disorder that leads to early death due to immunodeficiency, leukemia, and lymphoma. Given the underlying immune dysfunction and predisposition to hematologic cancers, bone marrow transplantation (BMT) or hematopoietic stem cell transplantation (HSCT) has emerged as a potential therapeutic strategy in pediatric patients with A-T. Therefore, longer follow-ups are needed to assess associated risks, side effects, procedures, and eligibility criteria. This systematic review aims to fill this gap by consolidating evidence from different parts of the

II Brazilian Consensus on the use of human immunoglobulin in patients with primary immunodeficiencies.

In the last few years, new primary immunodeficiencies and genetic defects have been described. Recently, immunoglobulin products with improved compositions and for subcutaneous use have become available in Brazil. In order to guide physicians on the use of human immunoglobulin to treat primary immunodeficiencies, based on a narrative literature review and their professional experience, the members of the Primary Immunodeficiency Group of the Brazilian Society of Allergy and Immunology prepared an updated document of the 1st Brazilian Consensus, published in 2010. The document presents new know

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… variable immunodeficiency with predominantly B-cell number and function abnormalities D83.2 - Common variable immunodeficiency with anti-B or T-cell autoantibodies D83.8 - Other common variable immunodeficiencies OBTAINING HUMAN IMMUNOGLOBULIN FOR THERAPEUTIC USE IN BRAZIL Treatment with human Ig … Efficacy of intravenous immunoglobulin in the prevention of pneumonia in patients with common variable immunodeficiency. J Allergy Clin Immunol. 2002;109(6):1001–1004. doi: 10.1067/mai.2002.124999. 97. Maarschalk-Ellerbroek LJ, Hoepelman IM, Ellerbroek PM. …

Secondary antibody deficiencies: what's around the corner?

Secondary antibody deficiencies (SADs) are characterized by impaired humoral immunity, which can cause recurrent and severe infections. Several factors may contribute to SAD development, making it difficult to establish a clear etiological classification. This heterogeneity also leads to clinical variability, further complicating patient management and treatment strategies. Various diagnostic and therapeutic algorithms are often adapted from those used in primary antibody deficiencies, potentially resulting in under- or over-treatment. Key points include the decision to initiate Immunoglobulin

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… Impaired vaccine response is an important item according to the main diagnostic criteria for common variable immunodeficiency and is useful in deciding treatment strategy ( 181 – 183 ). … In PADs, especially in Common Variable Immunodeficiency, IgRT is a lifelong treatment. Generally, the proper monthly dosage is about 400–600 mg/kg but may be increased/decreased depending on trough level IgG values and infectious complications. …

Esid 2014 oral presentations.

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… ESID-0719 IL21 Deficiency Results in Early-Onset Inflammatory Bowel Disease and Common Variable Immunodeficiency–Like Disease E. Salzer 1 , A. Kansu 2 , H. Sic 3 , P. Majek 4 , A. Ikinciogullari 5 , F.E. Dogu 5 , N.K. Serwas 1 , E. Santos-Valente 1 , W.F. Pickl 6 , I. Bilic 1 , S.A. Ban 1 , Z. … ESID-0806 CLEC16A Associates with Human Common Variable Immunodeficiency and Influences Murine B Cell Survival and Function J. Li 1 , S. Jørgensen 2 , S.M. Maggadottir 1 , M. Bakay 1 , K. Warnatz 3 , J. Glessner 1 , U. Salzer 3 , R.E. Schmidt 4 , E. Resnick 5 , S. Goldacker 3 , M. Buchta 3 , T. …

Decoding Immunodeficiencies with Artificial Intelligence: A New Era of Precision Medicine.

Primary and secondary immunodeficiencies comprise a wide array of illnesses marked by immune system abnormalities, resulting in heightened vulnerability to infections, autoimmunity, and cancers. Notwithstanding progress in diagnostic instruments and an enhanced comprehension of the underlying pathophysiology, delayed diagnosis and underreporting persist as considerable obstacles. The implementation of artificial intelligence into clinical practice has surfaced as a viable method to enhance early detection, risk assessment, and management of immunodeficiencies. Recent advancements illustrate ho

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… The burden of common variable immunodeficiency disorders: A retrospective analysis of the European Society for Immunodeficiency (ESID) registry data Orphanet J. Rare Dis. 2018 13 201 30419968 10.1186/s13023-018-0941-0 PMC6233554 33. Seth N. Tuano K.S. Chinen J. … Lymphoproliferative disease and cancer among patients with common variable immunodeficiency Leuk. Res. 2015 39 389 396 10.1016/j.leukres.2015.02.002 25711943 95. Riaz I.B. Faridi W. Patnaik M.M. Abraham R.S. …

Development of an Expert-Based Scoring System for Early Identification of Patients with Inborn Errors of Immunity in Primary Care Settings - the PIDCAP Project.

Early diagnosis of inborn errors of immunity (IEIs) has been shown to reduce mortality, morbidity, and healthcare costs. The need for early diagnosis has led to the development of computational tools that trigger earlier clinical suspicion by physicians. Primary care professionals serve as the first line for improving early diagnosis. To this end, a computer-based tool (based on extended Jeffrey Modell Foundation (JMF) Warning Signs) was developed to assist physicians with diagnosis decisions for IEIs in the primary care setting. Two expert-guided scoring systems (one pediatric, one adult) wer

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… Health-related quality of life in adult patients with common variable immunodeficiency disorders and impact of treatment. J Clin Immunol. 2017;37(5):461–75. 10. Elsink K, van Montfrans JM, van Gijn ME, Blom M, van Hagen PM, Kuijpers TW, et al. … Electronic health record signatures identify undiagnosed patients with common variable immunodeficiency disease. Sci Transl Med. 2024;16(745):eade4510. 26. Kumar B, Zetumer S, Swee M, Endelman ELK, Suneja M, Davis B. …

MicroRNA changes with macro potential contribute to secondary immunodeficiency in chronic lymphocytic leukemia during epstein barr virus reactivation.

Chronic lymphocytic leukemia (CLL) is characterized by immune dysfunctions driven by miRNA deregulation and the activation of immune checkpoint pathways, which contribute to disease progression and secondary immunodeficiency (SID). This study examines the interplay between miRNA expression profiles, Epstein-Barr virus (EBV) reactivation, and immune checkpoint pathways in the context of small intestine disease (SID) development in chronic lymphocytic leukemia (CLL). Patients were stratified into groups based on the presence of SID and EBV reactivation. Comprehensive analyses included miRNA prof

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… Mertowska P Immune checkpoint pathway expression in lymphocyte subpopulations in patients with common variable immunodeficiency and chronic lymphocytic leukemia Cancers 2023 15 5184 10.3390/cancers15215184 37958359 PMC10649987 Mertowska, P. et al. … Immune checkpoint pathway expression in lymphocyte subpopulations in patients with common variable immunodeficiency and chronic lymphocytic leukemia. Cancers 15 , 5184 (2023). 37958359 10.3390/cancers15215184 PMC10649987 9. …

Advancements in Immunology and Microbiology Research: A Comprehensive Exploration of Key Areas.

Immunology and microbiology research has witnessed remarkable growth and innovation globally, playing a pivotal role in advancing our understanding of immune mechanisms, disease pathogenesis, and therapeutic interventions. This manuscript presents a comprehensive exploration of the key areas in immunology research, spanning from the utilisation of bacterial proteins as antibody reagents to the intricate realms of clinical immunology and disease management. The utilisation of bacterial immunoglobulin-binding proteins (IBPs), including protein A (SpA), protein G (SpG), and protein L (SpL), has r

The obesity paradox in critically ill patients: a causal learning approach to a casual finding.

BackgroundWhile obesity confers an increased risk of death in the general population, numerous studies have reported an association between obesity and improved survival among critically ill patients. This contrary finding has been referred to as the obesity paradox. In this retrospective study, two causal inference approaches were used to address whether the survival of non-obese critically ill patients would have been improved if they had been obese.MethodsThe study cohort comprised 6557 adult critically ill patients hospitalized at the Intensive Care Unit of the Ghent University Hospital be

Implementing calculated globulin screening for immunodeficiencies: The benefits and challenges.

There is a need for pragmatic and cost-effective methodologies to facilitate earlier diagnosis of patients with primary and secondary immunodeficiencies, who often experience diagnostic delays that impact their quality of life, morbidity, and mortality. This review article will consider the utility of calculated globulin (CG) as a simple and unbiased screening tool for immunodeficiencies. We review literature indicating that CG can serve as an inexpensive and routinely performed proxy for immunoglobulin levels due to their established correlation. We also set out a workflow for the implementat

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… The onset of common variable immunodeficiency (CVID), the most common PAD found in adults ( 12 ), has been reported in patients 3 to 79 years of age, with a median age of onset of 23 and 28 years in males and females, respectively ( 11 ). … Economic burden of common variable immunodeficiency: Annual cost of disease . Expert Rev. Clin. Immunol. 11 : 681 – 688 . 10.1586/1744666X.2015.1029457 25804338 20. Yadav , H. , D. Shah , S. Sayed , S. Horton , and L.F. Schroeder . 2021 . …

Human herpes virus co-infection is associated with mortality in HIV-negative patients with Pneumocystis jirovecii pneumonia.

The purpose of this investigation was to characterize the management and prognosis of severe Pneumocystis jirovecii pneumonia (PJP) in human immunodeficiency virus (HIV)-negative patients. An observational cohort study of HIV-negative adults with PJP documented by bronchoalveolar lavage (BAL) through Gomori-Grocott staining or immunofluorescence, admitted to one intensive care unit (ICU) for acute respiratory failure, was undertaken. From 1990 to 2010, 70 patients (24 females, 46 males) were included, with a mean age of 58.6 ± 18.3 years. The mean Simplified Acute Physiology Score (SAPS)-II wa

Comparison of clinical and prognostic characteristics of patients with classic and AIDS-related Kaposi sarcoma: A single-center experience.

Kaposi sarcoma (KS) is a rare disease caused by human herpesvirus-8 (HHV-8) infection, that occurs in the elderly and more often in men. We aimed to compare the clinical and prognostic features of patients with classic and AIDS-related KS who have been followed in our clinic over the last 10 years and to present real-life data in first-line treatment. We retrospectively reviewed the medical records of 73 patients with KS, 57 with classic KS and 16 with acquired immunodeficiency syndrome (AIDS)-related KS, who were followed up at our clinic between January 2014 and December 2023. Information an