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Waldenström macroglobulinemia: clinical and immunological aspects, natural history, cell of origin, and emerging mouse models.

Waldenström macroglobulinemia (WM) is a rare and currently incurable neoplasm of IgM-expressing B-lymphocytes that is characterized by the occurrence of a monoclonal IgM (mIgM) paraprotein in blood serum and the infiltration of the hematopoietic bone marrow with malignant lymphoplasmacytic cells. The symptoms of patients with WM can be attributed to the extent and tissue sites of tumor cell infiltration and the magnitude and immunological specificity of the paraprotein. WM presents fascinating clues on neoplastic B-cell development, including the recent discovery of a specific gain-of-function

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… These cells were first identified in patients with common variable immunodeficiency (CVID), especially in those with splenomegaly and granulomatous disease [ 199 ], but later found to be abnormally elevated in peripheral tissues, such as the bronchoalveolar space [ 198 ]. …

Rare Extraoral Plasmablastic Lymphoma in the Context of HIV and Toxoplasmosis: A Detailed Case Analysis With Therapeutic Insights.

Plasmablastic lymphoma (PBL), a rare aggressive B-cell malignancy linked to HIV, is increasingly noted in extraoral sites beyond its traditional oral cavity presentation. This case report details a 48-year-old HIV-positive woman with prior cerebral toxoplasmosis, presenting with facial swelling, pain, proptosis, and dysphagia despite antiretroviral therapy and cotrimoxazole prophylaxis (CD4 120 cells/μL, suboptimal viral suppression). Imaging identified a sinonasal mass with nasopharyngeal involvement and lymphadenopathy, with immunohistochemistry confirming PBL (MUM1+, weakly CD79a+, CD20-, C

Yellow nail syndrome: a review.

Yellow nail syndrome (YNS; OMIM 153300, ORPHA662) is a very rare disorder that almost always occurs after 50 years of age but a juvenile or familial form has also been observed. YNS is diagnosed based on a triad associating yellow nail discoloration, pulmonary manifestations (chronic cough, bronchiectasia, pleural effusion) and lower limb lymphedema. Chronic sinusitis is frequently associated with the triad. YNS etiology remains unknown but a role of lymphatic impairment is usually evoked. YNS is more frequently isolated but may be associated in rare cases with autoimmune diseases, other clini

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… Gupta et al. reported lymphopenia in two YNS patients (one with common variable immunodeficiency) with low percentages of CD4 + T cells, high percentages of CD8 + T cells and severe naïve CD4 + and CD8 + T-cell deficits responsible for muted T-cell responses to antigens. … A patient with common variable immunodeficiency treated with subcutaneous immunoglobulin mounted good responses in terms of frequency of infections, lymphedema and pleural effusions [ 70 ]. …

Abstracts from the ISSAID 2021 Periodic Congress : Virtual. May - Dec 2021

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T-cell Immunotherapies and the Role of Nonclinical Assessment: The Balance between Efficacy and Pathology.

Gene-engineered T-cell therapies have the potential to revolutionize the treatment of cancer. These therapies have shown exceptional clinical efficacy specifically in the field of B-cell malignancies and the first products (Kymriah™ and Yescarta™) have recently been approved in the United States for specific indications. The power of these treatments is also linked with a distinct set of toxicities both predicted and unpredicted, including off-tumor activity, cytokine release syndromes, and neurotoxicity, occasionally with fatal consequences. As these therapies begin to reach more patients, it

Haematological Diseases in the Tropics

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Shortage of plasma-derived medicinal products: what is next? narrative literature review on its causes and counteracting policies in Italy.

Introduction: This paper describes the peculiarities of the plasma-derived medicinal product (PDMP) market and illustrates the results of a review of the literature on policies aimed at counteracting the shortage of PDMPs. Characteristics of PDMPs: Plasma is primarily used for the industrial production of blood products (80%). The demand for PDMPs, particularly immunoglobulins (IGs), is increasing. However, the production of PDMPs is complex, long (7-12 months), and expensive, accounting, according to US estimates, for 57% of the total costs of PDMPs compared to 14% for small molecules. PDMP m

Ход работы сессии 31st Европейский педиатрический конгресс по педиатрии: часть 1.

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In vitro расширила человека CD4+CD25+ регуляционные T клетки, подавляющие распространение Т клеток.

Регулирующие Т клетки (Tregs) оказались критически важными в балансе между автоиммунизацией и терпимостью и были связаны с несколькими аутоиммунными заболеваниями человека. Однако небольшое число трегов в периферии крови ограничивает их терапевтический потенциал. Поэтому мы разработали протокол, который позволит расширить "Трегз" при сохранении их подавляющей активности. Мы изолировали клетки CD4+CD25 hi от человеческой периферической крови и расширили их in vitro в присутствии anti-CD3 и anti-CD28 Магнитные кситы и высокие концентрации внешнего интерлейкина (IL) -2. Траги были эффективно расш

Summary of the National Advisory Committee on Immunization (NACI) Statement: Updated recommendations on herpes zoster vaccination for adults who are immunocompromised.

BACKGROUND: Herpes zoster (HZ), or shingles, results from the reactivation of latent varicella-zoster virus and poses a significant health burden and immunocompromised adults are at higher risk of HZ and its complications. In 2018, the recombinant zoster vaccine (RZV, Shingrix®) was strongly recommended by the National Advisory Committee on Immunization (NACI) for immunocompetent adults aged 50 years and older. Since then, evidence has accumulated on the use of RZV in immunocompromised adults and in 2021, Health Canada expanded the authorization of RZV to adults 18 years of age and older who a

The War Is on: The Immune System against Glioblastoma-How Can NK Cells Drive This Battle?

Natural killer (NK) cells are innate lymphocytes that play an important role in immunosurveillance, acting alongside other immune cells in the response against various types of malignant tumors and the prevention of metastasis. Since their discovery in the 1970s, they have been thoroughly studied for their capacity to kill neoplastic cells without the need for previous sensitization, executing rapid and robust cytotoxic activity, but also helper functions. In agreement with this, NK cells are being exploited in many ways to treat cancer. The broad arsenal of NK-based therapies includes adoptiv

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… Ebbo et al. (2016) [ 96 ], analyzing 457 patients with common variable immunodeficiency (CVID) in a 9-year follow-up multicenter cohort study, showed that the degree of NK lymphopenia did not correlate with the incidence of cancer; they hypothesized that the lack of correlation could be attributed to …

Nonlinear Association Between Calculated Globulin Levels and 28-Day Mortality in Patients with Sepsis: A Retrospective Cohort Study.

BackgroundSepsis remains a significant global health challenge, causing approximately 11 million deaths annually. The calculated globulin (CG) level, which is derived from total protein and albumin levels, plays crucial roles in the immune response and inflammation. However, the relationship between the CG level and sepsis mortality remains unexplored.MethodsThis retrospective cohort study analyzed sepsis patients from the eICU Collaborative Research Database. The primary outcome was 28-day ICU mortality. The relationship between the CG level and mortality was examined via generalized additive

The Genetics and Epigenetics of 22q11.2 Deletion Syndrome.

Chromosome 22q11.2 deletion syndrome (22q11.2del) is a complex, multi-organ disorder noted for its varying severity and penetrance among those affected. The clinical problems comprise congenital malformations; cardiac problems including outflow tract defects, hypoplasia of the thymus, hypoparathyroidism, and/or dysmorphic facial features. Additional clinical issues that can appear over time are autoimmunity, renal insufficiency, developmental delay, malignancy and neurological manifestations such as schizophrenia. The majority of individuals with 22q11.2del have a 3 Mb deletion of DNA on chrom

Exploring the Significance of Immune Checkpoints and EBV Reactivation in Antibody Deficiencies with Near-Normal Immunoglobulin Levels or Hyperimmunoglobulinemia.

This study delves into the intricate landscape of primary immunodeficiencies, with a particular focus on antibody deficiencies characterized by near-normal immunoglobulin levels or hyperimmunoglobulinemia. Contrary to the conventional focus on genetic dysregulation, these studies investigate the key roles of immune checkpoints, such as PD-1/PD-L1, CTLA-4/CD86, and CD200R/CD200, on selected subpopulations of T and B lymphocytes and their serum concentrations of soluble forms in patients recruited for the studies in healthy volunteers. In addition, the studies also show the role of Epstein-Barr

Progressive Multifocal Leukoencephalopathy after Administration of Front-Line Obinutuzumab and Venetoclax in a Patient with Chronic Lymphocytic Leukemia: Case Report.

INTRODUCTION: Progressive multifocal leukoencephalopathy (PML) is a rare and often fatal demyelinating disease of the central nervous system caused by the reactivation of the John Cunningham virus (JCV) in immunocompromised patients. Obinutuzumab is type II anti-CD 20 antibody, used in combination with venetoclax for chronic lymphocytic leukemia (CLL). CASE PRESENTATION: A 75-year-old female patient was diagnosed with PML after a short course of obinutuzumab and venetoclax started for a newly diagnosed CLL. There was no history of hepatitis B, C, or human-immunodeficiency virus. Quantitative

Сравнительное исследование распространенности усталости у лиц с заболеваниями: систематический обзор и мета-анализ.

Усталость является одним из наиболее распространенных симптомов у лиц с заболеваниями или нарушениями, значительно влияющих на качество жизни (КЖ) и прогноз заболеваний. Настоящее исследование направлено на всестороннее сравнение особенностей усталости в широком спектре заболеваний. Мы систематически искали данные в базах данных PubMed и Cochrane Library с момента их создания до 31 марта 2021 года и провели метаанализ для получения точных оценок. Анализы проводились по классификации заболеваний, полу и степени усталости (умеренная и тяжелая), а качество исследований оценивалось с использование

Clinician-Reported Respiratory Viral Testing and Intended Management of Community-Acquired Respiratory Viral Infections in Haematology and Haematopoietic Cell Transplantation: An International Exploratory Survey.

Background: Community-acquired respiratory viruses (CARVs) cause significant morbidity and mortality in patients with haematological malignancies and those undergoing haematopoietic cell transplantation (HCT). Despite this, clinical management remains variable. This study evaluated clinicians' awareness, diagnostic practices, and treatment approaches to major respiratory viruses in this high-risk population. Methods: An international electronic survey using non-probability convenience sampling was conducted among haematology, oncology, intensive care and emergency medicine physicians. Four rea

Hemophagocytic Lymphohistiocytosis (HLH) in Patients with Tick-Borne Illness: A Scoping Review of 98 Cases.

Hemophagocytic lymphohistiocytosis (HLH) secondary to tick-borne infections is a rare but potentially life-threatening syndrome. We performed a scoping review according to PRISMA guidelines to systematically analyze the existing literature on the topic. A total of 98 patients were included, with a mean age of 43.7 years, of which 64% were men. Most cases, 31%, were reported from the USA. Immunosuppression was present in 21.4%, with the most common cause being previous solid organ transplantation. Constitutional symptoms were the most common, observed in 83.7% of the patients, while fever was r

Management of refractory autoimmune hemolytic anemia after allogeneic hematopoietic stem cell transplantation: current perspectives.

Autoimmune hemolytic anemia (AIHA) is increasingly observed after allogeneic hematopoietic stem cell transplantation (allo-HSCT), with a reported incidence between 4% and 6%. The disease is generally severe and refractory to standard therapy, with high mortality, and there are neither defined therapies, nor prospective clinical trials addressing the best treatment. Most of the knowledge on the therapy of AIHAs derives from primary forms, which are highly heterogeneous as well, further complicating the management of post-allo-HSCT forms. The review addresses the risk factors associated with pos

Respiratory syncytial virus in hematopoietic cell transplant recipients and patients with hematologic malignancies.

In the USA and other western nations, respiratory syncytial virus is one of the most commonly encountered respiratory viruses among patients who have been diagnosed with a hematologic malignancy or who have undergone a stem cell transplant. Multiple studies have been performed to evaluate the complications associated with respiratory syncytial virus infections. Other studies have evaluated therapeutic agents and strategies in which these agents can be used. There have also been numerous reports of outbreaks in bone marrow transplant units and oncology wards, where infection control measures ha