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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

Rapid Onset of B12 Deficiency in the Setting of Worsening Multiple Myeloma: Correlations between B12 Deficiency and Multiple Myeloma.

A 67-year-old female with a relapse of multiple myeloma after being in remission for approximately 2 years following autologous stem cell transplant presented with worsening pancytopenia, over a three-month period. There were an increase in her monoclonal spike at 3.13 g/dL on serum protein electrophoresis, low serum B12 levels, and positive intrinsic factor antibodies. Three months before, she had normal B12 levels and a significantly lower monoclonal spike of 1.07 g/dL. She was diagnosed with B12 deficiency with pernicious anaemia in the setting of her worsening myeloma. Multiple myeloma (MM

Исследование реальных наблюдений за инфекциями у лиц, проходящих лечение острелизумабами, в связи с множественным склерозом.

ИСТОРИЯ ВОПРОСА: Anti-CD20 Моноклональные антитела в настоящее время являются обычным средством лечения склероза (MS). Rituximab, ocrelizumab и outatumumumab были связаны с зависящим от дозы риском гипогаммаглобулинамии, однако ее актуальность в клинической практике остается неопределенной. ЦЕЛИ: Изучение показателей инфицирования с течением времени в реальной когорте людей, которые лечатся окролизумабами, и их связи с сывороткой иммуноглобулин. DESIGN: Исследование по наблюдению 152 :: лица, получающие ocrelizumab для MS 5.6 Годы (среднее 2.7 США).Результаты: Среднегодовые (SD) изменения в им

Patient-reported outcomes with subcutaneous immunoglobulin in secondary immunodeficiency.

Subcutaneous (SCIG) and intravenous immunoglobulin (IVIG) replacement are both used to prevent infections in patients with secondary immunodeficiency (SID). Compared with IVIG, SCIG has fewer systemic side effects and, additionally, facilitates home-based treatment. Shared decision-making practice should include discussion of aspects such as patient preference as well as the associated risks and benefits of treatment. We review the available evidence for the use of SCIG treatment in patients with SID, focusing on patient-reported outcomes (PROs). In most studies, there were improvements to hea

Clinical outcomes of immunoglobulin treatment for patients with secondary antibody deficiency: Data from the Ontario immunoglobulin treatment case registry.

BackgroundDespite the increasing number of cases of secondary antibody deficiency (SAD) and immunoglobulin (Ig) utilization, there is a paucity of data in the literature on clinical and patient-reported outcomes in this population.ObjectiveTo describe immunoglobulin utilization patterns, clinical and patient-reported outcomes in patients with SAD on immunoglobulin replacement therapy (IgRT).MethodsA cross-sectional study of patients with secondary antibody deficiency enrolled in the Ontario Immunoglobulin Treatment (ONIT) Case Registry from June 2020 to September 2022 was completed. Demographi

Comparison of different sets of immunological tests to identify treatable immunodeficiencies in adult bronchiectasis patients.

BACKGROUND: The reported prevalence of immunodeficiencies in bronchiectasis patients is variable depending on the frequency and extent of immunological tests performed. European Respiratory Society guidelines recommend a minimum bundle of tests. Broadening the spectrum of immunological tests could increase the number of patients diagnosed with an immunodeficiency and those who could receive specific therapy. The primary objective of the present study was to assess the performance of different sets of immunological tests in diagnosing any, primary, secondary or treatable immunodeficiencies in a

The Production Processes and Biological Effects of Intravenous Immunoglobulin.

Immunoglobulin is a highly diverse autologous molecule able to influence immunity in different physiological and diseased situations. Its effect may be visible both in terms of development and function of B and T lymphocytes. Polyclonal immunoglobulin may be used as therapy in many diseases in different circumstances such as primary and secondary hypogammaglobulinemia, recurrent infections, polyneuropathies, cancer, after allogeneic transplantation in the presence of infections and/or GVHD. However, recent studies have broadened the possible uses of polyclonal immunoglobulin showing that it ca

Challenges in the Role of Gammaglobulin Replacement Therapy and Vaccination Strategies for Hematological Malignancy.

Patients with chronic lymphocytic leukemia (CLL) and multiple myeloma (MM) are prone to present with antibody production deficits associated with recurrent or severe bacterial infections that might benefit from human immunoglobulin (Ig) (IVIg/SCIg) replacement therapy. However, the original IVIg trial data were done before modern therapies were available, and the current indications do not take into account the shift in the immune situation of current treatment combinations and changes in the spectrum of infections. Besides, patients affected by other B cell malignancies present with similar i

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… 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 … deficiency Severe combined immunodeficiency (SCID) Secondary immunodeficiencies | Transplant acceptors Chronic lymphocytic leukaemia High-risk newborns AIDS in children Kawasaki’s disease Other disorders | Idiopathic thrombocytopenic purpura (acute) Haemophilia, autoantibodies against the factor VIII …

ENPP1/CD203a-targeting heavy-chain antibody reveals cell-specific expression on human immune cells.

ENPP1/CD203a is a membrane-bound ectonucleotidase capable of hydrolyzing ATP, cGAMP and other substrates. Its enzymatic activity plays an important role in the balance of extracellular adenine nucleotides and the modulation of purinergic signaling, in soft tissue calcification, and in the regulation of the cGAS/STING pathway. However, a detailed analysis of ENPP1 surface expression on human immune cells has not been performed. Here, we selected VHH domains from human ENPP1-immunized alpacas to generate heavy-chain antibodies targeting ENPP1, and analyzed cell surface expression on all circulat

Приобретение гепариновых антикоагулянтов, связанных с множественной миеломой: three доклады и обзор литературы.

Множественная миелома (ММ) часто связана с аномалиями коагуляции, которые могут проявляться либо в тромботических, либо в геморрагических осложнениях. Среди них приобщившиеся гепаринноподобные антикоагуланты (HLA), приводящие к крайнему увеличению тромбинного времени (TT) и спонтанному кровотечению, являются крайне редкими. Раннее признание и надлежащее вмешательство имеют решающее значение для терпеливых результатов. Представляемая информация: Three (больные)two мужчины и мужчины one женщины в возрасте 56–63 ) с подтвержденным MM, спонтанным кровотечением: уральное мукосальное кровотечение, ж

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

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

Risk factors and characteristics of blood stream infections in patients with newly diagnosed multiple myeloma.

BackgroundPatients with multiple myeloma are generally immune-compromised either due to pronounced depression in primary antibody responses or because of anti-myeloma therapy. Infection is a major risk factor for early deaths among these patients. The impact of blood stream infections (BSI) on newly diagnosed myeloma patients has been less studied. We aimed to study the incidence and risk factors of BSI within 3 months after diagnosis of multiple myeloma in a tertiary referral center.MethodsBetween November 2002 and December 2008, consecutive patients with multiple myeloma in Taipei Veterans G

IgG replacement in multiple myeloma.

T cell engagers (TCE) such as chimeric antigen receptor (CAR) T cell therapy and bispecific antibodies (BiAbs) for the treatment of multiple myeloma (MM) have significantly improved clinical outcomes, but have also raised awareness for ensuing post-treatment secondary immunodeficiency and hypogammaglobulinemia (HG). As patients with MM live longer, recurrent infections become a significant component of therapy-associated morbidity and mortality. Treatment of HG with immunoglobulin G replacement therapy (IgG-RT) has been a mainstay of the primary immunodeficiency (PI) world, and extrapolation t

Immunodeficiencies in Adults: Key Considerations for Diagnosis and Management.

Immunodeficiencies in adults are increasingly recognized yet often remain underdiagnosed, leading to significant morbidity from recurrent infections, autoimmunity, and malignancy. Both primary immunodeficiencies (PIDs), now known as inborn errors of immunity (IEI), and secondary immunodeficiencies (SIDs) contribute to immune dysfunction in adults. Although SIDs are more common in adults due to factors like medications, malignancies, metabolic disorders, chronic conditions, and protein-losing conditions, IEI-particularly common variable immunodeficiency (CVID)-can also manifest in adulthood wit

The interplay of immunity and growth: a case of combined variable immunodeficiency and growth hormone deficiency.

INTRODUCTION: Common variable immunodeficiency (CVID) is one of the more common immunodeficiencies seen in clinical practice with a complex disease pathology; while growth hormone deficiency (GHD) is a disorder characterized by complete or relative absence of the human growth hormone. CASE PRESENTATION: This case report presents a 13-year-old female patient with a long history of recurrent respiratory tract and ear infections, along with a notable failure to hit her developmental milestones early in the second decade of her life. The diagnosis was based on a thorough investigation of serum im

Subcutaneous immunoglobulin replacement for treatment of humoral immune dysfunction in patients with chronic lymphocytic leukemia.

BackgroundPatients with chronic lymphocytic leukemia (CLL) experience hypogammaglobinemia and non-neutropenic infections. In this exploratory proof of concept study, our objective was to determine the prevalence of humoral immunodeficiency in patients with CLL and serum IgG ≥ 400 mg/dL, and to evaluate the efficacy of subcutaneous immunoglobulin (SCIG) in this population.Patients and methodsPatients with CLL with serum IgG ≥ 400 mg/dL were evaluated for serum IgG, IgM, IgA, along with pre/post vaccine IgG titers to diphtheria, tetanus, and Streptococcus pneumoniae. Patients with evidence of hu

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

Immunodeficiencies and CFTR dysfunction: results from a systematic screening in a cohort of adults with cystic fibrosis and CFTR-related disorders.

BackgroundImmunodeficiencies (IDs) are conditions caused by immune system dysfunctions which predispose to chronic infections. Cystic fibrosis (CF) patients are characterized by the presence of bronchiectasis filled with hyper-viscous secretions that constitute the ideal environment for infections. Although CF and IDs might share similarities in the pathophysiological mechanism of bronchiectasis development, they each offer different treatment options. We hypothesize that the introduction of a bundle of tests would increase the number of ID diagnoses among adults with Cystic Fibrosis Transmemb