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Case Report: First occurrence of smoldering multiple myeloma in activated phosphoinositide 3-Kinase δ syndrome.

Activated phosphoinositide 3-kinase δ syndrome (APDS) is a newly described inborn error of immunity. Heterozygous mutations in the PI3Kδ catalytic subunit p110δ (PIK3CD) or regulatory subunit p85α (PIK3R1) lead to APDS1 and APDS2, respectively. Clinical manifestations include lymphoproliferation, recurrent respiratory tract infections, herpesvirus infections, enteropathy, elevated IgM, autoimmunity, and increased susceptibility to tumors, particularly lymphoma. Smoldering multiple myeloma (SMM), an asymptomatic clonal plasma cell disorder considered a pre-malignant entity, may progress to symp

Hemophagocytic Lymphohistiocytosis Associated with Synergistic Defects of AP3B1 and ATM Genes: A Case Report and Literature Review.

Hemophagocytic lymphohistiocytosis (HLH) is an overwhelming immune system activation that manifests as hyperinflammation and life-threatening multiple organ failure. However, the clinical manifestations of the systemic inflammatory response in sepsis and fulminant cytokine storm caused by HLH macrophage activation are very similar and difficult to distinguish. HLH triggered by two novel gene defects manifesting with multiorgan dysfunction syndrome (MODS) and distributive shock has not been reported. A 14-year-old male patient was hospitalized with a high fever, his condition deteriorated rapid

Prognostic Factors on the Graft-versus-Host Disease-Free and Relapse-Free Survival after Adult Allogeneic Hematopoietic Stem Cell Transplantation.

The cure of hematologic disorders by allogeneic hematopoietic stem cell transplantation (HSCT) is often associated with major complications resulting in poor outcome, including graft-versus-host disease (GVHD), relapse, and death. A novel composite endpoint of GVHD-free/relapse-free survival (GRFS) in which events include grades 3-4 acute GVHD, chronic GVHD requiring systemic therapy, relapse, or death is censored to completely characterize the survival without mortality or ongoing morbidity. In this regard, studies attempting to identify the prognostic factors of GRFS are quite scarce. Thus,

The role of therapeutic plasma exchange in plasma cell disorders.

Therapeutic plasma exchange (TPE) is an extracorporeal technique where patient's plasma containing pathogenic substances is separated and removed from the whole blood, while the cellular component is returned to the patient mixed with replacement solution via an apheresis machine. Due to its ability to remove pathogenic substances from plasma including immunoglobulins, TPE has proven efficacious in the management of various disorders across different medical disciplines, including plasma cell dyscrasias, which are characterized by the abundant secretion of non-functional immunoglobulins produc

Primary Aggressive Oral Lymphomas (PAOL): A Narrative Review of Diagnosis, Molecular Features, Therapeutic Approaches, and the Integrated Role of Dentists and Hematologists.

Primary aggressive oral lymphomas (PAOL) are a rare subset of extranodal non-Hodgkin lymphomas arising in the oral cavity without evidence of other systemic involvement at diagnosis. PAOL accounts for only about 2-3% of all lymphomas. They most commonly belong to aggressive B-cell subtypes such as Diffuse large B-cell lymphoma (DLBCL) and plasmablastic lymphoma (PBL), with occasional cases of Burkitt lymphoma and T-cell/NK-cell lymphomas. Clinically, these malignancies often present with non-specific symptoms (e.g., swelling, pain, ulceration, tooth mobility) that mimic benign dental condition

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… Similarly, organ transplant recipients on chronic immunosuppressive therapy can develop EBV-driven oral lymphoproliferative disorders [ 14 ]. Overall, immunodeficiency (HIV infection or iatrogenic) is a significant contributor to oral lymphoma pathogenesis [ 11 , 12 , 13 , 14 ]. … Posttransplant Lymphoproliferative Disorder: Otolaryngological Manifestations and Management Otolaryngol. Head Neck Surg. 2017 157 750 759 10.1177/0194599817707208 28535360 15. de Oliveira E.M. de Cáceres C.V.B.L. Fernandes-Rodrigues C.I. Penafort P.V.M. Legarrea J.M.A. Gomes N.R. Pontes H.A.R. …

Waldenström macroglobulinemia treatment algorithm 2018.

Waldenström macroglobulinemia is often an indolent disorder, and many patients are candidates for observation with careful monitoring. For symptomatic patients, one must distinguish between those patients whose symptoms are related to immunologic manifestations associated with the IgM monoclonal protein and those that have symptoms related to progressive marrow and nodal infiltration with lymphoplasmacytic lymphoma. In Waldenström macroglobulinemia, the driver for therapy in the majority of patients is progressive anemia, secondary to bone marrow replacement by lymphoplasmacytic lymphoma. Rece

Exploring the health benefits of outdoor exercise for cancer survivors: a systematic review of more than 700 individuals.

BackgroundCancer's rising incidence and growing survivor population underscore the need for strategies to enhance health and quality of life. Outdoor physical activity (PA) settings may provide unique benefits, yet evidence in this context is scarce. This systematic review aims to evaluate the impact of outdoor PA and exercise interventions on the health and well-being of cancer survivors.MethodsA systematic search was conducted in PubMed, Web of Science, and PsycINFO databases from their inception until April 23, 2024. Studies included randomized controlled trials (RCTs) and non-RCTs involvin

Immunotherapy in hematologic malignancies: achievements, challenges and future prospects.

The immune-cell origin of hematologic malignancies provides a unique avenue for the understanding of both the mechanisms of immune responsiveness and immune escape, which has accelerated the progress of immunotherapy. Several categories of immunotherapies have been developed and are being further evaluated in clinical trials for the treatment of blood cancers, including stem cell transplantation, immune checkpoint inhibitors, antigen-targeted antibodies, antibody-drug conjugates, tumor vaccines, and adoptive cell therapies. These immunotherapies have shown the potential to induce long-term rem

An Unusual Case of Extracavitary/Solid Variant Primary Effusion Lymphoma With Associated Hemophagocytic Lymphohistiocytosis.

Primary effusion lymphoma (PEL) is a rare, aggressive large B-cell lymphoma variant that is invariably associated with human herpesvirus 8 (HHV8), predominantly in human immunodeficiency virus (HIV)-infected patients, and its oncogenicity is often augmented by coinfection with Epstein-Barr virus. It typically presents as a serous effusion in body cavities without detectable solid tumors. The extracavitary variant of PEL may represent a diagnostic challenge. A 37-year-old man with HIV/acquired immunodeficiency syndrome (AIDS) was transferred to our hospital for evaluation of a mediastinal mass

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… Human herpesvirus 8 and lymphoproliferative disorders. Mediterr J Hematol Infect Dis. 2018;10(1):e2018061. doi: 10.4084/MJHID.2018.061. 29. Guillet S, Gerard L, Meignin V, Agbalika F, Cuccini W, Denis B, Katlama C. et al. …

Mucocutaneous Findings in Hematolymphoid Neoplasms: An Observational Study.

BackgroundCutaneous manifestations of hematological neoplasms can be divided into three broad categories - direct infiltration, paraneoplastic conditions, and those due to the treatment of hematological cancers.ObjectivesTo study the frequency and patterns of mucocutaneous manifestations in patients with hematolymphoid neoplasms and those due to chemotherapy.Materials and methodsThis was an observational study done with 172 patients. Categorization of mucocutaneous manifestations was done into malignancy-associated and chemotherapeutic drugs-associated and data was analyzed.ResultsOut of a tot

Oral arsenic trioxide for treating acute promyelocytic leukaemia: Implications for its worldwide epidemiology and beyond.

This account describes how orally administered Arsenic-trioxide (ATO) therapy influences the epidemiology of acute promyelocytic leukaemia (APL), and how the experience that ensued may expand the indications for oral ATO as a treatment for diseases/disorders other than APL. Over the last two decades, experience with APL patients in Hong Kong treated with an oral regimen comprising ATO, all-trans retinoic acid (ATRA), and ascorbic acid (also known as "AAA") has confirmed a dramatic improvement in overall survival. Over that period, there has been an estimated 60-fold increase in the prevalence

Гематологические маньяки маскируются как ревматологические заболевания: серия дел и обзор.

RATALE: Гематологическая недостаточность может имитировать ревматологические заболевания, создавая серьезную диагностическую проблему из-за перекрывающихся клинических особенностей. В настоящем исследовании подчеркивается 5 - случаи гематологических маньяков, представляющих собой ревматологические расстройства, и обсуждаются связанные с этим диагностические сложности. БОЛЕЕ ТРУДЯЩИЕСЯ: 64 в адрес 78, с различными рематологическими симптомами, включая полиартрит, васкулит, явление Рейно, и такими систематическими симптомами, как потеря веса, усталость и ночной пот. В ходе первоначальной эксперт

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… Flow cytometry of the peripheral blood was nondiagnostic for lymphoproliferative disorders, and the remainder of the autoimmune and viral serologies were unremarkable. …

Pleuropulmonary Changes Induced by Drugs in Patients with Hematologic Diseases

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… The drug is mainly used to treat malignant lymphomas of B cell lineage, post-transplantation lymphoproliferative disorders, bullous pemphigoid, and rheumatoid arthritis. Incidence of pulmonary toxicity is 0.03–8% [ 378 ]. …

Риски гематологических обледенений у пациентов, прошедших лечение от твердых опухолей, связанных с использованием пегилизованных гранулоцитов-стимулирующих факторов: 2005.

Широкое применение пегилизованных гранулоцитов (PGCS), в частности pegfilgrastim и липегфилграстим, в целях сокращения числа случаев нейтрализации женских половых органов у пациентов, проходящих химиотерапию. Вместе с тем по-прежнему сохраняется озабоченность по поводу долгосрочной безопасности ПХГФ, в частности по поводу риска вторичных гематологических заболеваний. Цель настоящего исследования заключалась в оценке распространенности вторичных гематологических заболеваний у пациентов, которые лечились от твердых опухолей с помощью PGCSF в одном учреждении 2005 в адрес 2019. Ретроспективный ан

Daratumumab and Nelarabine Treatment as Salvage Therapy for T-Lymphoblastic Lymphoma: A Case Report.

T-cell lymphoblastic lymphoma is an uncommon lymphoid neoplasm in adults, although more frequent in children and teenagers, that often affects the mediastinum and bone marrow, requiring intensive chemotherapy protocols. Its prognosis is poor if a cure is not achieved with first-line treatments. We present a case report of a 19-year-old man diagnosed with this type of lymphoma due to significant respiratory distress and a mediastinal mass. He received treatment according to the hyper-CVAD regimen, with a complete metabolic response. However, seven months later a new mediastinal growth was obser

Case report: Relapsed/refractory extranodal natural killer/T-cell lymphoma nasal type with extensive central nervous system involvement.

Background: Extranodal natural killer/T-cell lymphoma (ENKL) is a rare subtype of mature T and natural killer cell lymphomas associated with Epstein-Barr virus. Case: A 20-year-old presented with severe neurological symptoms and was diagnosed with stage IV ENKL, nasal type, with CNS involvement. Overall, the patient received nine treatment lines, including chemotherapy, craniospinal irradiation, allogeneic stem cell transplant (alloSCT), donor lymphocyte infusions, and novel agents (Nivolumab, Daratumumab, Thalidomide, Lenalidomide, virus-specific T cells) combined with intrathecal chemotherap

Bendamustine: safety and efficacy in the management of indolent non-hodgkins lymphoma.

Bendamustine (Treanda, Ribomustin) was recently approved by the US Food and Drug Administration (FDA) for treatment of patients with rituximab refractory indolent lymphoma and is expected to turn into a frontline therapy option for indolent lymphoma. This compound with amphoteric properties was designed in the former Germany Democratic Republic in 1960s and re-discovered in 1990s with multiple successive well-designed studies. Bendamustine possesses a unique mechanism of action with potential antimetabolite properties, and only partial cross-resistance with other alkylators. Used in combinatio

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… Several well-designed and well-supervised studies in recent years have provided exciting results that has enthused great interest in the potential role of bendamustine for treatment of lymphoproliferative disorders. …

Более низкий риск множественного склероза у пациентов с хроническим гепатитом С: общенациональное исследование по регистрации населения.

Множественный склероз (MS) — иммунопосредованное неврологическое заболевание, вызывающее демиэлирование. Этиология неизвестна, однако пациенты с предыдущей вирусной инфекцией, такой, как вирус Эпштейн-Барр, оказались в более опасном положении с точки зрения развития MS. С другой стороны, лица, инфицированные ВИЧ, имеют более низкий риск развития НР. Вирус гепатита С (ГКВ) в основном заражает печень, но пациенты с ГЦВ могут испытывать несколько внеэптических проявлений, и исследования показали связь с несколькими аутоиммунными заболеваниями, такими, как нейропатия и миелит. Настоящее исследован

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… It is also expressed on B cells as part of a costimulatory complex, and patients with HCV-related lymphoproliferative disorders have elevated serum concentrations of B-cell-activating factor (BAFF) [ 8 ]. …

Iron Sucrose: A Wealth of Experience in Treating Iron Deficiency.

Iron deficiency and iron-deficiency anemia are associated with increased morbidity and mortality in a wide range of conditions. In many patient populations, this can be treated effectively with oral iron supplementation; but in patients who are unable to take or who do not respond to oral iron therapy, intravenous iron administration is recommended. Furthermore, in certain conditions, such as end-stage kidney disease, chronic heart failure, and inflammatory bowel disease, intravenous iron administration has become first-line treatment. One of the first available intravenous iron preparations i

The Expanding Field of Secondary Antibody Deficiency: Causes, Diagnosis, and Management.

Antibody deficiency or hypogammaglobulinemia can have primary or secondary etiologies. Primary antibody deficiency (PAD) is the result of intrinsic genetic defects, whereas secondary antibody deficiency may arise as a consequence of underlying conditions or medication use. On a global level, malnutrition, HIV, and malaria are major causes of secondary immunodeficiency. In this review we consider secondary antibody deficiency, for which common causes include hematological malignancies, such as chronic lymphocytic leukemia or multiple myeloma, and their treatment, protein-losing states, and side

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… Subcutaneous immunoglobulin in lymphoproliferative disorders and rituximab-related secondary hypogammaglobulinemia: a single-center experience in 61 patients. Haematologica (2014) 99:1101–6. 10.3324/haematol.2013.101261 19. Gunther G, Dreger B. …