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Is dosing of therapeutic immunoglobulins optimal? A review of a three-decade long debate in europe.

The consumption of immunoglobulins (Ig) is increasing due to better recognition of antibody deficiencies, an aging population, and new indications. This review aims to examine the various dosing regimens and research developments in the established and in some of the relevant off-label indications in Europe. The background to the current regulatory settings in Europe is provided as a backdrop for the latest developments in primary and secondary immunodeficiencies and in immunomodulatory indications. In these heterogeneous areas, clinical trials encompassing different routes of administration,

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… Common variable immunodeficiency: clinical and immunological features of 248 patients . Clin Immunol ( 1999 ) 92 : 34 – 48 . 10.1006/clim.1999.4725 10413651 12 Jolles S Kaveri SV Orange J Intravenous immunoglobulins. Current understanding and future directions . … Clinical picture and treatment of 2212 patients with common variable immunodeficiency . …

Bridging the Gap in Secondary Antibody Deficiencies: Current Evidence and Unmet Needs in Diagnosis and Management with Immunoglobulin Replacement.

Secondary antibody deficiency (SAD) represents a substantial yet under-recognised global healthcare burden. It is more prevalent than primary antibody deficiency, but frequently under-diagnosed and variably managed worldwide. Prompt diagnosis is often hindered by insufficient awareness among clinicians, lack of global consensus on screening/monitoring for SAD among at-risk patients, inadequate clinical immunology services and lack of standardised referral pathways/protocols. Management practices vary widely, with little international agreement, particularly regarding threshold to initiate immu

Use of Immunoglobulin Replacement Therapy in Clinical Practice: A Review.

Immunoglobulins (Igs) are produced by B lymphocytes and play a key role in humoral immunity. Igs are classified into five isotypes (IgG, IgA, IgM, IgE, and IgD). Their primary function is to recognize and bind to foreign antigens. When Igs bind to antigens, they facilitate phagocytosis and promote clearance mediated by other immune cells. It is an essential component in protecting the host from outside pathogens. Hypogammaglobulinemia predisposes an individual to severe and recurrent infections. Therefore, replacement therapy is recommended to maintain optimal Ig level. In addition, Igs can mo

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… Common variable immunodeficiency (CVID), the most common symptomatic IEI in adults, is characterized by hypogammaglobulinemia, impaired specific antibody responses, and typically, normal B cell counts. … Infection outcomes in patients with common variable immunodeficiency disorders: relationship to immunoglobulin therapy over 22 years. J Allergy Clin Immunol . 2010;125:1354–1360.e4. 16. Orange J, Belohradsky B, Berger M, et al. …

Clinical Associations with Hospital Escalation Among COVID-19 Patients Receiving Remdesivir in a Hospital-at-Home Service: A Real-World Cohort Study.

Background/Objectives: Hospital-at-home (HaH) programs expanded hospital capacity during the COVID-19 pandemic, but data on which HaH patients receiving intravenous (IV) remdesivir may require hospital escalation is limited. We therefore aimed to explore clinical characteristics associated with escalation to inpatient care. Methods: Single-center, retrospective cohort of adults with confirmed COVID-19 who received IV remdesivir via a HaH service was employed (September 2020-September 2024). Primary outcome was hospital escalation. Associations between baseline variables and escalation were ass

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… Condition | n (%) Hematologic | 11 (14.1) Chronic lymphocytic leukemia † | 3 (3.8) Myelodysplastic syndrome | 2 (2.6) Common variable immunodeficiency | 1 (1.3) Acute myeloid leukemia | 1 (1.3) Follicular lymphoma | 2 (2.6) Marginal zone lymphoma | 1 (1.3) Multiple myeloma | 1 (1.3) Immunosuppressive …

The Burden of Human Metapneumovirus (hMPV) Disease in Older and High-Risk Adults in Developed Countries: A Systematic Literature Review.

IntroductionHuman metapneumovirus (hMPV) causes respiratory infections in individuals of all age groups. While the evidence on hMPV epidemiology is growing, the hMPV burden in adults has not been synthesised. We conducted a critical review of published evidence on the burden of hMPV in older adults and adults with comorbidities.MethodsArticles reporting the burden of hMPV disease in adults ⩾50 years or ⩾18 years with comorbidities in developed countries were searched in Embase and MEDLINE (1/January/2000-12/June/2023) databases. Observational and interventional studies on community-based or me

Azathioprine for remission maintenance in anti-interferon-γ autoantibody-associated immunodeficiency syndrome- a retrospective single-center cohort study.

BACKGROUND: Anti-interferon-γ autoantibody (Anti-IFN-γ Aab) disease is associated with frequent recurrent infections. Adjunctive immunosuppressive treatment, including rituximab, can achieve disease remission. However, disease relapses and recurrent infections occur after immunosuppressive drugs are discontinued. Azathioprine (AZA) is used for maintenance therapy in many autoimmune diseases with few adverse effects. This study aimed to assess the effectiveness of AZA in maintaining clinical remission in patients with Anti-IFN-γ Aab disease. METHODS: This retrospective observational study was

Assessment of Treatment Outcomes and Associated Factors Among Pediatric Patients With Burkitt Lymphoma at Kenyatta National Hospital.

BACKGROUND: In developing countries, the treatment outcomes of Burkitt lymphoma are poor due to the poorly equipped healthcare systems. In addition, there is limited comprehensive data within the African continent, including Kenya, about the outcomes of treatment for this cancer. AIMS: To assess treatment outcomes and variables associated with an increased risk of death from disease progression or treatment-related toxicities among Burkitt lymphoma pediatric patients at the Kenyatta National Hospital (KNH). METHODS AND RESULTS: A retrospective one-arm cohort study was conducted to examine th

An Observational Analysis of a Large Cohort of Pneumocystis jirovecii Pneumonia.

BACKGROUND: Pneumocystis jirovecii pneumonia (PCP) is increasingly seen in patients who are non-HIV immunocompromised hosts, yet modern risk factors and outcomes are poorly recognized and defined. METHODS: A retrospective review was conducted of adult patients who met criteria for proven or probable PCP from 2017 to 2024 across a large academic multicenter health system. The primary outcome was mortality among cohorts, defined by etiology of immunosuppression through univariate and age-adjusted logistic regression. Secondary analyses included trends in annual diagnoses and testing patterns.

Prognosis and treatment of plasmablastic lymphoma in the United States: a multicenter retrospective study.

Plasmablastic lymphoma (PBL) is a rare, aggressive AIDS-related lymphoma observed in patients with immunosuppressed states as well as in immunocompetent individuals. We sought to determine survival outcomes, prognostic factors, and optimal treatment regimens in a large, contemporary cohort of patients with PBL in the United States. We performed a multicenter, retrospective cohort study, including 344 patients diagnosed with PBL between 2005 and 2022. Patients were stratified into cohorts according to underlying immune status. Survival outcomes were calculated using Kaplan-Meier statistics, wit

Immunoglobulin replacement therapy in secondary hypogammaglobulinemia.

Immunoglobulin (Ig) replacement therapy dramatically changed the clinical course of primary hypogammaglobulinemias, significantly reducing the incidence of infectious events. Over the last two decades its use has been extended to secondary antibody deficiencies, particularly those related to hematological disorders as lymphoproliferative diseases (LPDs) and multiple myeloma. In these malignancies, hypogammaglobulinemia can be an intrinsic aspect of the disease or follow chemo-immunotherapy regimens, including anti-CD20 treatment. Other than in LPDs the broadening use of immunotherapy (e.g., ri

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… Common variable immunodeficiency unmasked by treatment of immune thrombocytopenic purpura with rituximab. BMC Hematol (2013) 13(1):4. 10.1186/2052-1839-13-4 34. Nishio M, Endo T, Fujimoto K, Yamamoto S, Obara M, Yamaguchi K, et al. …

Real-World Effectiveness, Safety, and Tolerability of Facilitated Subcutaneous Immunoglobulin 10% in Secondary Immunodeficiency Disease: A Systematic Literature Review.

Background: Secondary immunodeficiency disease (SID) is a complex, heterogeneous condition that occurs when extrinsic factors weaken the immune system. Expert consensus guidelines recommend immunoglobulin replacement therapy to manage immunoglobulin G (IgG) levels and mitigate severe, recurrent, and persistent infections. Hyaluronidase-facilitated subcutaneous immunoglobulin (fSCIG) 10% is a dual-vial unit of IgG and recombinant human hyaluronidase; the latter enables absorption of higher volumes of IgG than conventional subcutaneous therapies. Methods: For this systematic literature review, E

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… Tolerability has also been demonstrated in two patients with thrombocytopenia and common variable immunodeficiency who did not report any local hemorrhagic manifestations at the infusion site [ 52 ]. … Facilitated subcutaneous immunoglobulin (fSCIg) in autoimmune cytopenias associated with common variable immunodeficiency. Isr. Med. Assoc. J. 2017;19:420–423. 52. Danieli M.G., Pulvirenti F., Rocchi V., Morariu R., Quinti I. …

Study protocol for developing a patient-reported outcome measure for infection screening in hematological patients with secondary immunodeficiency.

BACKGROUND: Patients with chronic lymphocytic leukemia (CLL) or multiple myeloma (MM) frequently develop secondary immunodeficiencies (SID), on the basis of both their respective disease, as well as immunosuppressive therapies, leading to increased infection risks and impaired health-related quality of life (HRQOL). While timely infection detection is essential, outpatient symptom monitoring remains challenging. Electronic patient-reported outcome (ePRO) tools have shown promise for real-time symptom monitoring, enhancing early detection and management. We describe a study protocol for develop

Litigations for HIV Related Complications

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Prevalence of Vitamin D Deficiency in Ataxia-Telangiectasia: A Systematic Review and Single Arm Meta-Analysis.

Ataxia-telangiectasia (A-T) is a multisystem disorder in which nutritional and endocrine abnormalities are frequently reported. Vitamin D deficiency has been described in A-T cohorts, but its pooled prevalence within published A-T cohorts has not been quantified. We conducted a systematic review and single-arm meta-analysis to estimate the prevalence of vitamin D deficiency in A-T. We systematically identified observational studies reporting serum 25-hydroxyvitamin D 25(OH)D in individuals with A-T. The primary outcome was vitamin D deficiency prevalence using the study-defined threshold of 25

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… In a cross-sectional controlled study, Cruz et al. (2019) evaluated serum 25-hydroxyvitamin D [25(OH)D] levels in patients with Common Variable Immunodeficiency (CVID) and A–T. They found vitamin D deficiency in A–T and CVID patients. … Assessment of vitamin D status in common variable immunodeficiency or ataxia–telangiectasia patients. Allergol Immunopathol. 2019;47:499–505. 6. Prayle A, et al. G401 (P) Low vitamin d levels in children with ataxia-telangiectasia. BMJ Publishing Group Ltd.; 2017. 7. Nissenkorn A, et al. …

Critically Ill Immunosuppressed Host

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Vaccination of Immunocompromised Cats.

Immunocompromise is a common condition in cats, especially due to widespread infections with immunosuppressive viruses, such as feline immunodeficiency virus (FIV) and feline leukaemia virus (FeLV), but also due to chronic non-infectious diseases, such as tumours, diabetes mellitus, and chronic kidney disease, as well as treatment with immunosuppressive drugs, such as glucocorticoids, cyclosporins, or tumour chemotherapy. In this review, the European Advisory Board on Cat Diseases (ABCD), a scientifically independent board of experts in feline medicine from eleven European countries, discusses

Combined Immune Defect in B-Cell Lymphoproliferative Disorders Is Associated with Severe Infection and Cancer Progression.

B cell chronic lymphoproliferative diseases (B-CLPD) are associated with secondary antibody deficiency and other innate and adaptive immune defects, whose impact on infectious risk has not been systematically addressed. We performed an immunological analysis of a cohort of 83 B-CLPD patients with recurrent and/or severe infections to ascertain the clinical relevance of the immune deficiency expression. B-cell defects were present in all patients. Patients with combined immune defect had a 3.69-fold higher risk for severe infection (p = 0.001) than those with predominantly antibody defect. Inte

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… Serum Free Immunoglobulins Light Chains: A Common Feature of Common Variable Immunodeficiency? Front. Immunol. 2020 11 2004 10.3389/fimmu.2020.02004 32849664 PMC7431983 36. Chatsirisupachai K. Lagger C. de Magalhães J.P. …

Impact of COVID-19 on immunocompromised populations during the Omicron era: insights from the observational population-based INFORM study.

BackgroundImmunocompromised individuals are not optimally protected by COVID-19 vaccines and potentially require additional preventive interventions to mitigate the risk of severe COVID-19. We aimed to characterise and describe the risk of severe COVID-19 across immunocompromised groups as the pandemic began to transition to an endemic phase.MethodsCOVID-19-related hospitalisations, intensive care unit (ICU) admissions, and deaths (01/01/2022-31/12/2022) were compared among different groups of immunocompromised individuals vs the general population, using a retrospective cohort design and elec

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… “Stringently-defined immunocompromised” comprised a subset of the broadly-defined and included any individual with ≥1 of the following: Moderate to severe primary immunodeficiency: common variable immunodeficiency disease, severe combined immunodeficiency, DiGeorge syndrome, or Wiskott-Aldrich syndrome …

ENHANZE® drug delivery technology: a novel approach to subcutaneous administration using recombinant human hyaluronidase PH20

ENHANZE® drug delivery technology is based on the proprietary recombinant human hyaluronidase PH20 enzyme (rHuPH20; Halozyme Therapeutics, Inc.) that facilitates the subcutaneous (SC) delivery of co-administered therapeutics. rHuPH20 works by degrading the glycosaminoglycan hyaluronan (HA), which plays a role in resistance to bulk fluid flow in the SC space, limiting large volume SC drug delivery, dispersion, and absorption. Co-administration of rHuPH20 with partner therapies can overcome administration time and volume barriers associated with existing SC therapeutic formulations, and has been

Primary and Secondary Immunodeficiency Diseases in Oncohaematology: Warning Signs, Diagnosis, and Management.

Background: Immunodeficiencies (ID), in particular primary immunodeficiencies (PID), are often associated with haematological manifestations, such as peripheral cytopenias or lymphoproliferative syndromes. Early diagnosis and management have significant prognostic implications. Secondary immunodeficiencies (SID) may also be induced by oncohaematological diseases and their treatments. Haematologists and oncologists must therefore be aware of the association between blood disorders and cancer and ID, and be prepared to offer their patients appropriate treatment without delay. Our aim was to defi