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Периодическая эпистоксика как редкая презентация экстрамеуллярной мультимиеломы.

Множественная миелома, отличающаяся распространением клональных плазменных клеток за пределами костного мозга, связана с неблагоприятным прогнозом, особенно в тех случаях, когда она сопровождается цитогенетическими отклонениями высокого риска. С китайской стороны редко случается, и она может быть сопряжена с периодическими эпистазами или односторонними носовыми обструкциями, что создает диагностическое дублирование с подстрекательскими, сосудистыми, лимфоидными и эпителиальными синузами. Мы сообщаем о 54-летнем мужчине с восьмимесячной историей прогрессивной правосторонней носовой обструкции и

Рекомендации Международной группы по работе с миеломой по диагностике и лечению изолированных плазмоклеточных опухолей.

Одиночные плазмоклеточные опухоли (СП) — редкие локализованные опухоли клоновых плазмоклеток, либо в костной ткани (одиночная костная плазмоклеточная опухоль), либо в мягких тканях/экстраоссозных (экстромедуллярные) с отсутствием или минимальным проникновением в костный мозг (КМ) (<10% клоновых плазмоклеток по иммуногистохимии) и отсутствием признаков системного вовлечения или событий, определяющих миелому. Приблизительно 50% СП прогрессируют в симптоматическую миелому в течение 5 лет после начальной определенной локальной радиотерапии. Увеличение доступности улучшенных диагностических и монит

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… Adapted from Fend et al. 45 Abbreviations: BM, bone marrow; EBV, Epstein-Barr virus; EMM, extramedullary multiple myeloma; EMZL, extranodal marginal zone B-cell lymphoma; IGH: immunoglobulin heavy chain; LC, light chain; LPL, lymphoplasmacytic lymphoma; MM, multiple myeloma; PBL, plasmablastic lymphoma … Morphologic and immunohistochemical studies cannot always differentiate between plasmablastic lymphoma and SEMP; markedly increased proliferation rate, necrosis, and association with EBV infection, as well as aggressive clinical presentation, suggest plasmablastic lymphoma rather than SEMP; a rare EBV-positive …

Reviewing the Significance of Vitamin D Substitution in Monoclonal Gammopathies.

Vitamin D is a steroid hormone that is essential for bone mineral metabolism and it has several other effects in the body, including anti-cancer actions. Vitamin D causes a reduction in cell growth by interrupting the cell cycle. Moreover, the active form of vitamin D, i.e., 1,25-dihydroxyvitamin D, exerts various effects via its interaction with the vitamin D receptor on the innate and adaptive immune system, which could be relevant in the onset of tumors. Multiple myeloma is a treatable but incurable malignancy characterized by the growth of clonal plasma cells in protective niches in the bo

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… CYP27B1, together with VDR, is also expressed in other plasmablastic B-cell malignancies, such as plasmablastic lymphoma and diffuse large B-cell lymphoma. … These reports were confirmed by a different group of researchers, who studied plasmablastic lymphoma and myeloma cells more broadly [ 120 ]. …

Воздействие высокоэффективной терапии множественного склероза на клетки B.

Множественный склероз (MS) представляет собой хроническое воспалительное и нейродегенеративное аутоиммунное расстройство центральной нервной системы, характеризующееся демиэлированием и нейродегенерацией. Традиционно рассматриваемая как болезнь с Т-клетоклетками, ключевая роль В-лимфоцитов в его патогенезе через различные механизмы, способствующие воспалению и автоматической активности, получает все большее признание. Риск долгосрочной инвалидности у пациентов с НР может быть снижен за счет раннего начала лечения, в частности с помощью высокоэффективной терапии. Цель этого обзора состоит в том

Malignant Neoplasms Arising in the Cardiac Pacemaker Cavity: A Systematic Review.

Cancer is the abnormal proliferation of physiologically inadequate cells. Studies have identified the cardiac pacemaker pocket as a site of rare neoplasms. To evaluate the clinical outcomes, treatment, prognosis, and individualized management of tumors originating in the cardiac pacemaker pocket, a systematic review was conducted using case reports and case series available in the PubMed/Medline, Science Direct, Cochrane Central, LILACS, and Scientific Electronic Library Online (Scielo) databases. Pacemaker pocket tumors affected patients with a mean age of 72.9 years, with a higher incidence

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… Zarifi et al. [ 28 ] described a case of plasmablastic lymphoma affecting the PP of a 100-year-old male patient after a period of 10 years from implantation until the onset of symptoms, as observed in Table 1 below. Table 1 Identification of articles included in the study. … An enlarging pacemaker pocket: A case report of a plasmablastic lymphoma arising as a primary tumor around a cardiac pacemaker and systematic literature review of various malignancies arising at the pacemaker pocket. J. Cardiol. Cases. 2017;17:41–43. doi: 10.1016/j.jccase.2017.09.006. 29. …

An unexpected cause of a swollen pacemaker pocket: a case report.

BACKGROUND: A complication originating from the pacemaker pocket after device implantation can most often be explained by a post-operative pocket haematoma, or, less frequently, by a pocket infection. Both conditions need immediate assessment, dedicated treatment, and specialized follow-up. In rare cases, however, a swollen pacemaker pocket has an alternative diagnosis, which is exemplified by the following case. CASE SUMMARY: A 70-year-old male patient had a-specific symptoms of fatigue, dyspnoea, and coughing for some weeks. He also noted an evident, new swelling of his pacemaker pocket sev

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… lymphoma , 14 plasmacytoma, 15 and multiple myeloma. 16 The detection of an active oncologic process, a primary tumour or a metastasis in a pacemaker pocket is a rare phenomenon. … An enlarging pacemaker pocket: A case report of a plasmablastic lymphoma arising as a primary tumor around a cardiac pacemaker and systematic literature review of various malignancies arising at the pacemaker pocket. J Cardiol Cases 2018;17:41–43. 15. Hamaker WR, Lindell ME, Gomez AC. …

CAR-T cell therapies in autoimmune rheumatic diseases: a brief report on the clinical trial landscape, current status, and future perspectives.

Autoimmune rheumatic diseases (ARDs) are chronic inflammatory disorders where B cells play a key role. Traditional B-cell-targeted therapies have limitations, whereas CAR-T-cell therapy, which aims for a broader reset of the B-cell compartment by targeting B-cell surface markers such as CD19 or B-cell maturation antigen (BCMA), has unique advantages. Currently, most CAR-T cell trials for ARDs are in the early stages, with 64.29% (36/56 trials) of studies being phase I trials and only 7.14% (4/56 trials) progressing to phase II trials, primarily focusing on conditions, such as systemic lupus er

Targeting CD38 Across Autoimmune and Plasma Cell-Driven Immune-Mediated Diseases: Rationale, Clinical Evidence, and Emerging Therapeutic Landscape.

Autoimmune diseases remain a major cause of chronic morbidity despite substantial advances in targeted immunomodulatory therapies. In many autoantibody-mediated conditions, disease refractoriness and relapse are driven by long-lived plasma cells, which are largely resistant to conventional immunosuppression and upstream B cell-directed strategies. CD38, a surface molecule highly expressed on plasmablasts and plasma cells and functionally involved in immunometabolic regulation, has emerged as a promising therapeutic target to overcome this limitation. Clinical interest in anti-CD38 therapy has

Multiple myeloma with intracranial and spinal intradural metastasis: A case report.

Multiple myeloma is a hematopoietic cancer that is multicentric and most commonly involves the spine. Multiple myeloma with extraosseous and intradural involvement is an extremely rare condition. Here we present a rare case of spinal multiple myeloma with intracranial and spinal intradural metastasis causing lumbar spinal nerve compression. We present a 60-year-old woman with progressive weakness of the lower limbs for several weeks. Spinal magnetic resonance imaging (MRI) showed a leptomeningeal tumor with nodularity spreading within the cauda equina. Examination of the brain using MRI showed

Secondary Hemophagocytic Syndrome in a Patient with Plasma Cell Myeloma and CNS Involvement Treated with Lenalidomide.

We present an extremely rare case report of a 29-year-old multiple myeloma patient with central nervous system involvement and secondary hemophagocytic lymphohistiocytosis (HLH). We observed that HLH was presumably triggered by the immunomodulatory drug-lenalidomide. HLH is frequently misdiagnosed or underdiagnosed. As HLH requires immediate treatment, our report emphasizes the need to consider HLH in the differential diagnosis when the condition of a patient receiving chemotherapy rapidly deteriorates and an infectious etiology is excluded. We furthermore discuss the pathogenesis of HLH, with

Новые горизонты для мультислерозовой терапии: 2025 и дальше.

Прогресс, достигнутый в борьбе с множественным склерозом (МС), представляет собой one о больших успехах современной молекулярной медицины. Развитие методов лечения с повышением избирательности и безопасности, руководствуясь достижениями в понимании фундаментальной иммунологии, нейробиологии, генетики и причин этой болезни, расширило традиционный акцент на лечении, добавив реальные возможности для профилактики и лечения. Здесь мы резюмируем последние достижения, которые вместе превратили эту болезнь из наиболее часто встречающегося в западном мире криппера на ее место сегодня как условие, в кот

Plasmablastic plasmacytoma of the rectum and anal canal in a patient with ulcerative colitis.

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B-cells in pulmonary arterial hypertension: friend, foe or bystander?

There is an unmet need for new therapeutic strategies that target alternative pathways to improve the prognosis of patients with pulmonary arterial hypertension (PAH). As immunity has been involved in the development and progression of vascular lesions in PAH, we review the potential contribution of B-cells in its pathogenesis and evaluate the relevance of B-cell-targeted therapies. Circulating B-cell homeostasis is altered in PAH patients, with total B-cell lymphopenia, abnormal subset distribution (expansion of naïve and antibody-secreting cells, reduction of memory B-cells) and chronic acti

Spotlight on quetiapine in bipolar depression.

Because of its relapsing-remitting nature, patients with multiple myeloma [1,2] require extended therapy with chemotherapy, novel agents, autologous stem cell transplants (ASCT), monoclonal antibodies and/or immune therapies for optimal outcomes. Attrition during treatment can negatively impact long-term outcomes and factors influencing the same may be important clues to achieve optimal patient outcomes. This study aims to examine attrition rates and the factors influencing them.We analysed 244 consecutive newly diagnosed patients of multiple myeloma from January 2017 to December 2017 register

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… Patients diagnosed with solitary plasmacytoma, plasmablastic lymphoma, smoldering myeloma, MGUS, amyloidosis, plasma cell leukemia, POEMS syndrome and relapsed cases were excluded from the study. … Results Data of 244 patients were analysed for demographic data, treatment characteristics, overall survival, and progression free survival, excluding patients diagnosed with solitary plasmacytoma, plasmablastic lymphoma, smoldering myeloma, MGUS, amyloidosis, plasma cell leukemia, POEMS syndrome, and …

Pleural effusion and multiple myeloma - more than meets the eye: A case report.

Multiple myeloma (MM) accounts for 1% of all cancers. It consists of malignant proliferation of plasma cells, which is often associated with hypersecretion of a monoclonal protein. Pleural effusion (PE) in MM is not an uncommon finding, comprising about 6-14% of patients with MM. The most common causes of MM-associated PE are congestive heart failure, renal failure, parapneumonic effusion and amyloidosis. In <1% of cases, the effusion is a direct result of MM, designated as myelomatous PE (MPE). MPE is usually a diagnosis of exclusion and carries a poor prognosis. Therefore, efforts should be

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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… Morphologic and immunophenotypic overlap with other aggressive lymphomas, particularly plasmablastic lymphoma, ALK-positive large B-cell lymphoma, anaplastic plasmacytoma, and HHV8-positive large B-cell lymphoma, may challenge accurate diagnosis; the combination of positive CD138 and HHV8 and negative …

A rare primary pericardial DLBCL masquerading as an unexplained malignant pleural effusion in an elderly woman: A case report.

IntroductionPrimary cardiac lymphoma (PCL) is an extremely rare and fatal heart neoplasm. Primary cardiac non-Hodgkin lymphoma (NHL) is uncommon, considering the rarity of pericardial diffuse large B-cell lymphoma (DLBCL) with advanced pleural metastasis.Case presentationWe reported an 86-year-old female with primary pericardial DLBCL diagnosed initially by pleural effusion cytology. The chest imaging study revealed multiple pericardial lobulated infiltrative masses and epicardial invasion. Subsequently, she underwent an emergent pericardial window with a pericardial mass biopsy. The final his

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… The most common diagnostic lymphoma subtype is DLBCL, but Burkitt lymphoma, T-cell lymphomas, small lymphocytic lymphoma, and plasmablastic lymphoma also occur [ 1 , 6 ]. Pericardial involvement with secondary pleural effusion is extremely rare [ 7 ]. … However, in the literature, Burkitt's lymphoma, T-cell lymphomas, small lymphocytic lymphoma, and plasmablastic lymphoma could also occur [ 1 , 6 ]. Cardiac and pericardial lymphomas involvement is extremely rare, accounting for 0.5% of cardiac involvement and 1% of extranodal NHL. …

Clinical and Biological Characteristics of Medullary and Extramedullary Plasma Cell Dyscrasias.

Background: Extramedullary plasma cell (PC) disorders may occur as extramedullary disease in multiple myeloma (MM-EMD) or as primary extramedullary plasmocytoma (pEMP)/solitary osseous plasmocytoma (SOP). In this study, we aimed to obtain insights into the molecular mechanisms of extramedullary spread of clonal PC. Methods: Clinical and biological characteristics of 87 patients with MM-EMD (n = 49), pEMP/SOP (n = 20) and classical MM (n = 18) were analyzed by using immunohistochemistry (CXCR4, CD31, CD44 and CD81 staining) and cytoplasmic immunoglobulin staining combined with fluorescence in s

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… All 4 patients with only focal extramedullary relapse were treated locally again (resection and/or irradiation) while all 7 patients with relapse/progression to MM (or plasmablastic lymphoma in one patient) were treated systemically. …

Chimeric Antigen Receptor T-Cell Revolution Remodeling Immunity to Conquer Autoimmune Disease.

Chimeric antigen receptor T (CAR-T) cell therapy, a groundbreaking technology in tumor immunotherapy, has demonstrated unprecedented potential in the field of autoimmune diseases in recent years. This article provides a systematic review of the developmental trajectory and core concepts of CAR-T-cell therapy in autoimmune diseases, emphasizing its conceptual evolution from traditional "killing" strategies to "precision immune remodeling". Leveraging multitarget approaches (e.g., CD19 and B-cell maturation antigen) and chimeric autoantigen receptor technology, it achieves efficient elimination

Chimeric antigen receptors: "CARs" in the fast lane for rheumatology.

PURPOSE OF REVIEW: Recent advances in hematology-oncology have pioneered cell-mediated elimination of pathologic B-cell populations employing chimeric antigen receptor (CAR) T cells. In this review, we discuss recent adoption of CAR-T treatment for severe refractory autoimmune disease. We highlight unique aspects of the autoimmune model and review current clinical data regarding treatment of rheumatologic disease. RECENT FINDINGS: To date, several CAR-Ts are FDA approved for Multiple Myeloma and B-cell malignancies and have demonstrated extraordinary clinical responses in refractory disease.