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IL-22 способствует возникновению хронической гаммагерпесвирусной инфекции, поддерживая реакцию микробного центра.

Гаммагерпесвирусы, включая вирус Эпштейн-Барр (EBV) и герпесвирус, связанный с Карпози (KSHV), создают скрытые инфекции на протяжении всей жизни, которые способствуют множественным заболеваниям раком и множественным склерозом. Эти вирусы колонизируют наивные клетки B и приводят в действие мощный, поликлонный герминальный центр, чтобы расширить латентный вирусный резервуар и установить пожизненную инфекцию в клетках памяти B. Несмотря на клиническое бремя этих вирусов, факторы, способствующие хронической инфекции, по-прежнему плохо определены. " Interleukin-22 " (IL-22) является крити

Clinical effects of tranexamic acid on bleeding tendency due to fibrinolytic activation of AL amyloidosis.

We report a case where tranexamic acid, which is an antifibrinolytic agent, was used to effectively treat bleeding tendency in a patient with immunoglobulin light chain (AL) amyloidosis. A male patient in his 80s without a history of bleeding disorders was admitted to our hospital for the examination of bleeding tendency and was diagnosed with a bleeding disorder due to AL amyloidosis. Blood tests revealed elevated plasmin-α2-plasmin inhibitor complex levels, suggesting fibrinolytic activation. Managing the bleeding was difficult; however, we suspected fibrinolytic activation associated with A

Замена иммуноглобулина: Понятия управления несколькими миеломами.

Immunoglobulin (Ig) - заместительная терапия (IGRT) - состоит из применения низкодозированных поликлонных иг человека для лечения первичной и вторичной гипогаммаглобулинемии, которая связана с периодическими инфекциями и иммунной дисфункцией. ИГРТ восстанавливает физиологические уровни антител и вызывает иммуномумудуляторный эффект путем укрепления клеток иммунного эффекта, тем самым уменьшая инфекцию. Здесь мы описываем фармакологию различных составов иг с уделением особого внимания их механизму действий как низкодозированный ИГРТ, включая прямой антимикробный эффект и иммунодуляторную функци

16th International Congress on Antiphospholipid Antibodies Task Force Report on Antiphospholipid Syndrome Treatment Trends.

Antiphospholipid syndrome (APS), an acquired autoimmune thrombophilia, is characterised by thrombosis and/or pregnancy morbidity in association with persistent antiphospholipid antibodies. The 16th International Congress on Antiphospholipid Antibodies Task Force on APS Treatment Trends reviewed the current status with regard to existing and novel treatment trends for APS, which is the focus of this Task Force report. The report addresses current treatments and developments since the last report, on the use of direct oral anticoagulants in patients with APS, antiplatelet agents, adjunctive ther

Real-world evidence from Japan in patients with hematological malignancies and secondary hypogammaglobulinemia treated with 20% subcutaneous immunoglobulin, Ig20Gly: A retrospective cohort study.

Secondary immunodeficiency (SID) and hypogammaglobulinemia have been reported to be related to an increase in various infections and poor outcome, and it is important to evaluate immunoglobulin replacement therapy (IgRT) at an appropriate timing. The objective of this study is to evaluate the safety and efficacy of glycine-stabilized 20% subcutaneous immunoglobulin (Ig20Gly) in Japanese patients with hematological malignancies accompanied by SID. The patients who were administered Ig20Gly at least once at Tottori Prefectural Central Hospital were enrolled in this study. Information about the p

When Iron Bites Back: Severe Thrombocytopenia After Intravenous Iron Sucrose Therapy (Venofer).

Iron deficiency anemia (IDA) commonly presents with normal or elevated platelet counts, while thrombocytopenia is rare. Intravenous iron sucrose (Venofer) is widely used and generally well tolerated. We report the case of a 71-year-old female with chronic IDA who developed severe thrombocytopenia following iron sucrose therapy. Baseline hemoglobin was 6.3 g/dL with a platelet count of 229 × 10³/µL. After two doses of intravenous iron, hemoglobin improved to 9.3 g/dL, but platelets rapidly declined to 11 × 10³/µL. Extensive workup excluded thrombotic microangiopathy, autoimmune disease, infecti

Association Between Common Variable Immunodeficiency and Pulmonary Amyloidosis: Review.

Background: Common variable immunodeficiency (CVID) is the most frequent symptomatic primary antibody deficiency, associated with recurrent infections, immune dysregulation, and non-infectious complications. Amyloidosis is a rare but severe complication with pulmonary involvement being exceptional. Objective: The aim of this study was to review reported cases of amyloidosis complicating CVID and present a unique case of pulmonary involvement. Methods: A literature research identified observational studies and case reports linking amyloidosis with CVID. Additionally, we describe a patient with

Secondary Immunodeficiency and Hypogammaglobulinemia with IgG Levels of <5 g/L in Patients with Multiple Myeloma: A Retrospective Study Between 2012 and 2020 at a University Hospital in China.

BACKGROUND Infections are the main cause of mortality and morbidity in multiple myeloma (MM) patients. However, adult immunodeficiency specialists in China are lacking, and the care of secondary immunodeficiency (SID) and the prognostic role of hypogammaglobulinemia in MM is unknown. MATERIAL AND METHODS MM patients (295) were retrospectively analyzed between January 2012 and 2020 in Zhejiang Provincial People's Hospital, Hangzhou Medical College. MM patients with immunoglobulin (Ig) G <5 g/L were defined as SID patients. The care of these patients and the prognostic role of IgG <5 g/L were an

Immunological consequences of CAR T-cell therapy: an analysis of infectious complications and immune reconstitution.

AbstractChimeric antigen receptor (CAR) T-cell therapy has demonstrated remarkable efficacy in treating relapsed and refractory (R/R) B-cell neoplasms, such as diffuse large B-cell lymphoma (DLBCL) and multiple myeloma (MM). Despite its success, the long-term effects and sequelae of CAR T cells on the immune system remain underexplored. This study presents a 1-year follow-up analysis of 52 patients (42 with R/R DLBCL and 10 with R/R MM) treated with anti-CD19- and B-cell maturation antigen-targeted CAR T cells, focusing on immune reconstitution and infectious complications. Our findings reveal

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

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… In brief, patients in the SID cohort were defined as having either serum IgG levels < 5.0 g/L, specific antibody failure, ICD-10-CM codes for non-familial hypogammaglobulinemia (D80.1), selective IgG deficiency (D80.3), or antibody deficiency with near-normal immunoglobulins (D80.6), and the presence …

Persistent Increase in Serum Alkaline Phosphatase in a Patient with Monoclonal Gammopathy of Undefined Significance.

We report the finding of alkaline phosphatase-immunoglobulin complex (macro-alkaline phosphatase (macro-ALP)) in a patient with persistently increased ALP activity. The identification of macro-ALP is important to rule out pathological causes of increased ALP activity and to avoid unnecessary diagnostic investigation. The patient was subsequently diagnosed with vitamin D deficiency, gallstone pancreatitis, and monoclonal gammopathy of undefined significance (MGUS). Macro-ALP can coexist with disease that can increase serum ALP activity. We report, for the first time, a case of macro-ALP in a pa

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

Clinical Aspects of B Cell Immunodeficiencies: The Past, the Present and the Future.

B cells and antibodies are indispensable for host immunity. Our understanding of the mechanistic processes that underpin how B cells operate has left an indelible mark on the field of clinical pathology, and recently has also dramatically reshaped the therapeutic landscape of diseases that were once considered incurable. Evaluating patients with primary immunodeficiency diseases (PID)/inborn errors of immunity (IEI) that primarily affect B cells, offers us an opportunity to further our understanding of how B cells develop, mature, function and, in certain instances, cause further disease. In t

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… Maglione et al. measured serum BCMA levels in 165 patients and discovered that serum BCMA was significantly reduced in severe primary antibody deficiencies such as XLA and CVID, compared with less profound antibody deficiencies such as selective IgA or mild IgG deficiency [ 78 ]. …

A PRoliferation-Inducing Ligand (APRIL) in the Pathogenesis of Immunoglobulin A Nephropathy: A Review of the Evidence.

A PRoliferation-Inducing Ligand (APRIL), the thirteenth member of the tumor necrosis factor superfamily, plays a key role in the regulation of activated B cells, the survival of long-lived plasma cells, and immunoglobulin (Ig) isotype class switching. Several lines of evidence have implicated APRIL in the pathogenesis of IgA nephropathy (IgAN). Globally, IgAN is the most common primary glomerulonephritis, and it can progress to end-stage kidney disease; yet, disease-modifying treatments for this condition have historically been lacking. The preliminary data in ongoing clinical trials indicate

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

Ассоциированные клетки B в аутоиммунных болезнях: патогенез и клинические последствия.

Будучи разнородным подмножеством клеток В, связанные с возрастом клетки В (ABC) имеют различные транскрипционные профили, процессы экстрафолликулярной дифференциации и множественные функции в автоматическом иммунитете. Сигналы TLR7 и TLR9 наряду с стимуляцией IFN-__ и IL-21 имеют важное значение для дифференциации ABC, которая также регулируется хемокиновыми рецепторами, включая CXCR3 и CCR2, и интегралами, включая CD11b и CD11c. С учетом их функций, связанных с поглощением и представлением антигенов, секрецией цитокина, а также активацией ячеек Т, ABC демонстрируют потенциал в прогнозе, диагн

Targeting B cells in IgA nephropathy: from pathogenic insight to therapeutic horizon.

Immunoglobulin A nephropathy (IgAN) remains the most common primary glomerulonephritis globally. Advances in mucosal immunology have illuminated the role of dysregulated B cell activity, galactose-deficient IgA1 (Gd-IgA1) and Gd-IgA1-specific antibody formation in driving disease pathogenesis. Therapeutic targeting of the mucosal immune system and, more specifically, B cell survival and differentiation pathways have entered late-stage development, offering a mechanistically guided approach to disease modification. Targeting of the gut-associated lymphoid tissue of the terminal ileum, B cell ac

Immunoglobulin replacement vs prophylactic antibiotics for hypogammaglobulinemia secondary to hematological malignancy.

AbstractImmunoglobulin replacement and prophylactic antibiotics are commonly used to prevent infections in patients with secondary hypogammaglobulinemia due to hematological malignancies but have never been directly compared. In this randomized controlled feasibility trial conducted in 7 hospitals in Australia and New Zealand, we enrolled patients with secondary hypogammaglobulinemia with either a history of recurrent/severe infection or an immunoglobulin G level <4 g/L. Participants were randomized in a 1:2 ratio to immunoglobulin (0.4 g/kg per 4 weeks IV) or daily antibiotics (trimethoprim-s

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

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