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Bone marrow fibrosis in newly diagnosed multiple myeloma and its correlation with clinicopathological factors.

BACKGROUND: Bone marrow fibrosis (BMF) severely impacts both the quality of life and the efficacy of diagnostic procedures. However, the correlation between BMF and clinicopathological features, cytogenetic changes, and prognosis of newly diagnosed multiple myeloma (NDMM) remains unclear. This study determined the incidence, patient characteristics, and clinical outcomes of patients with NDMM with BMF. METHODS: The clinical data, histological features, and clinical outcomes of patients with NDMM were collected. Reticular fiber staining was performed on the enrolled cases, and the degree of re

RVd и CyBord терапии ремоделируют созревание клеток B и изменяют иммунную и клональную архитектуру в множестве миеломы.

Множественная миелома (ММ) характеризуется неоднородностью плазменных клеток (ПХ) и изменениями в дифференциации В-клеток и иммунной регламентации. Хотя линалидомид/бортезомиб/дексатон (RV) и циклофосфамид/бортезомиб/дексамасон (CyBorD) являются клинически эффективными, их точное воздействие на созревание ПК/B-клеток остается неясным. МЕТОДЫ: Мы провели профилирование КИТОФ на образцах костного мозга из RVd (n = 47) и пациентов MM, проходящих лечение CyBorD (n = 15), каждый по сравнению с неочищенной когортой (n = 43). Внутри каждого терапевтического рукава ответные устройства (RV, n = 35; CyB

Солитарная экстрамедуллярная плазмоцитома почки у ребёнка: описание клинического случая.

ВВЕДЕНИЕ: Солитарная экстрамедуллярная плазмоцитома (SEP) — редкое злокачественное новообразование из плазматических клеток, преимущественно встречающееся у взрослых, чаще всего в области головы и шеи. Поражение почки у детей встречается крайне редко. Представлен случай солитарной экстрамедуллярной плазмоцитомы почки у ребёнка, иллюстрирующий диагностические сложности и особенности ведения пациента. ОПИСАНИЕ КЛИНИЧЕСКОГО СЛУЧАЯ: У 10-летнего мальчика отмечалась периодическая безболевая макрогематурия. При компьютерной томографии (CT) выявлено кистозно-солидное образование размером 4,8 × 4,7 ×

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… Importantly, plasmablastic lymphoma (PBL) should be distinguished: although they may highly overlap in morphology and immunophenotype, PBL typically demonstrates an extremely high proliferation index (Ki-67 > 90%), EBER positivity, and is often associated with an immunosuppressed clinical background … Plasmablastic lymphoma: current knowledge and future directions. Front Immunol. 2024;15:1354604. 14. Finsinger P, Grammatico S, Chisini M, Piciocchi A, Foà R, Petrucci MT. Clinical features and prognostic factors in solitary plasmacytoma. Br J Haematol. 2016;172(4):554–60. 15. …

Chimeric antigen receptor T-cell therapy in systemic lupus erythematosus: From B cell depletion to immune reprogramming.

Systemic lupus erythematosus (SLE) is a complex autoimmune disease in which current immunosuppressive and biologic therapies often fail to achieve durable remission in refractory patients. The emergence of chimeric antigen receptor T-cell (CAR-T) therapy has introduced a fundamentally new therapeutic concept, shifting therapeutic strategies from transient immune suppression toward the possibility of durable immune reprogramming. Recent studies suggest that the benefit of CAR-T therapy extends beyond depletion of autoreactive B cells. By depleting disease-associated immune cell populations and

Osteolytic Lesion of the Maxilla in an Undiagnosed Multiple Myeloma Patient Identified Incidentally by Cone Beam Computed Tomography.

BACKGROUND Multiple myeloma is a hematological malignancy characterized by monoclonal plasma cell proliferation. Jaw lesions are found in nearly 35% of patients with symptomatic myeloma, and lesions occur in the mandible more often than in the maxilla. However, maxillary or mandibular lesions are rarely found as a primary manifestation of the disease. This report describes a case of a 65-year-old Palestinian woman with lytic lesions in the maxilla due to undiagnosed multiple myeloma identified incidentally on cone beam computed tomography (CBCT). CASE REPORT A 65-year-old Palestinian woman pre

B Cells and Antibodies as Targets of Therapeutic Intervention in Neuromyelitis Optica Spectrum Disorders.

The first description of neuromyelitis optica by Eugène Devic and Fernand Gault dates back to the 19th century, but only the discovery of aquaporin-4 autoantibodies in a major subset of affected patients in 2004 led to a fundamentally revised disease concept: Neuromyelits optica spectrum disorders (NMOSD) are now considered autoantibody-mediated autoimmune diseases, bringing the pivotal pathogenetic role of B cells and plasma cells into focus. Not long ago, there was no approved medication for this deleterious disease and off-label therapies were the only treatment options for affected patient

Химерический антигенный рецептор T-клеточная терапия в нейроиммунных болезнях: механистическое обоснование, клинический опыт и проблемы с переводом.

Нейроиммунные заболевания представляют собой разнородную группу расстройств, при которых клетки В, плазменные удары, плазменные клетки, автоантитела и Т клетки поразному влияют на повреждение тканей. Хотя биологика B-клеток улучшила контроль за заболеваниями, некоторые пациенты по-прежнему сталкиваются с реанимацией, рецидивом после лечения или кумулятивной неврологической инвалидностью. Химерическая антигенная рецепторная T-клетка (CAR-T-клетка) является средством достижения более глубокого и потенциально устойчивого истощения определенных патогенных иммунных клеток. Этот описательный обзор в

FK506-Binding Protein 11 Is a Novel Plasma Cell-Specific Antibody Folding Catalyst with Increased Expression in Idiopathic Pulmonary Fibrosis.

Antibodies are central effectors of the adaptive immune response, widespread used therapeutics, but also potentially disease-causing biomolecules. Antibody folding catalysts in the plasma cell are incompletely defined. Idiopathic pulmonary fibrosis (IPF) is a fatal chronic lung disease with increasingly recognized autoimmune features. We found elevated expression of FK506-binding protein 11 (FKBP11) in IPF lungs where FKBP11 specifically localized to antibody-producing plasma cells. Suggesting a general role in plasma cells, plasma cell-specific FKBP11 expression was equally observed in lympha

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

Терапия химическая антигенная терапия у пациентов, страдающих диабетом, с множественными миеломами: 5-Case Серия.

Цимерная антигенная рецепторная (CAR) T-клетотерапия стала трансформативным лечением множественных гематологовых магнентов, включая множественные миеломы. Однако пациенты с хроническими заболеваниями почек, особенно больные, страдающие диализом, как правило, исключаются из клинических испытаний, что оставляет значительный пробел в доказательствах, касающихся безопасности и целесообразности лечения этих пациентов с помощью ЦАР-Т-клеток. К числу проблем, вызывающих озабоченность у этой группы пациентов, относятся потенциальная токсичность лимфоразрушающих химиотерапий и повышенная подверженность

Impact of siltuximab on patient-related outcomes in multicentric Castleman's disease.

Multicentric Castleman's disease (MCD) is a rare, widespread lymphoproliferative disorder and a life-threatening disease involving hyperactivity of the immune system, excessive proinflammatory cytokine release, immune cell proliferation, and organ system dysfunction. Interleukin-6 (IL-6) is a cytokine that plays a key role in the pathogenesis of MCD, as it is involved in the synthesis of acute-phase reactants and aids in the induction of B-cell proliferation. Siltuximab is an anti-IL-6 chimeric monoclonal antibody that acts as a novel treatment modality to bind to IL-6 with high affinity, thus

Positive Anti-nuclear Antibody in Patients with Polyclonal Hypergammaglobulinemia Suggests the Presence of Multiple Distinct Comorbidities.

Objective Polyclonal hypergammaglobulinemia (PHGG) is a classic problem in internal medicine; however, its conditions and diagnostic procedures have not been well studied. We therefore conducted a retrospective study to characterize the PHGG disease spectrum. Methods We included all patients who underwent serum protein electrophoresis (SPEP) at a hematology tertiary referral center during a five-year period. For these patients, globulin clonality was determined and clinical data were extracted from the records. Results Out of 209 consecutive cases of hypergammaglobulinemia demonstrated by SPEP

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… lymphoma | 1 (1)* | | | | | | Acquired aplastic anemia | 1 (1)* | | | | | | Warm autoimmune hemolytic anemia | 1 (1)* | | | | | | NK-large granular lymphocytic leukemia | 1 | | | | | | Iron deficiency anemia | 1 | | | | | | Polymorphic lymphoproliferative disorders arising … Plasmablastic lymphoma was detected on biopsy. She had been carefully followed for CMML for three years. With dementia, best supportive care was chosen. | 37.3 | 0.37 | Nd | CMML, plasmablastic lymphoma Case 13 | 56-year-old man was hospitalized for liver transplant for AIH. …

CAR T клеточная терапия в неврологических расстройствах, вызванных автоантибиозами: многообещающая стратегия.

Клеточная терапия химерного антигенного рецептора (CAR) Т становится перспективным подходом к неврологическим аутоиммунным расстройствам на базе клеток B, особенно тем, которые характеризуются патогенными автоантиорганизмами, направленными на ключевые нейронные структуры. Эти заболевания, включая расстройство спектрального спектра нейромиелита, миастения гравис, синдром Ламберта-Итона миастеника, болезнь, связанная с антителами MOG, энцефалит рецептора против NMDA, диацилглицирол губазы альфа антитела, ассоциируемый с энцефалитом, синдромом жесткого человека и множественным склерозом, могут бы

Clonotypic characterization defines B-cell drivers of clonal expansion and intratumor heterogeneity in IgM monoclonal gammopathies.

Waldenström macroglobulinemia (WM) and IgM monoclonal gammopathy of undetermined significance (MGUS) share the same cell of origin but differ in clonal size. Compared with other B-cell neoplasms, the lymphoplasmacytic clone in WM can be rather small, limiting our understanding of clonal expansion. We applied an integrative approach using single-cell RNA with B-cell receptor (BCR) sequencing, the assay for transposase-accessible chromatin, and whole-genome sequencing to characterize the tumor clone in patients with IgM MGUS, smoldering WM (SWM), and symptomatic WM (WM). IgM MGUS and low- or int

Solitary Hepatic Plasmacytoma With IgM Monoclonal Gammopathy: A Case Report.

Solitary extramedullary plasmacytoma (SEP) is a rare plasma cell neoplasm that typically arises in the upper aerodigestive tract but may occur in other organs, including the liver. IgM-secreting SEPs are exceedingly rare and can be challenging to differentiate from other lymphomas with similar presentations. We report a case of a 73-year-old female who presented with fatigue and cytopenias, later found to have a large hepatic mass. Biopsy revealed a kappa-restricted plasma cell neoplasm with strong CD138, BCL2, CD20, and PAX5 expression and absence of MYD88 L265P mutation via immunohistochemis

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… The key diagnostic challenge lies in distinguishing IgM-secreting plasmacytomas from WM, plasmablastic lymphoma (PBL), and extranodal marginal zone lymphoma (MZL). … Plasmablastic Lymphoma: A Systematic Review The Scientific World Journal 2011 March 11 687 696 10.1100/tsw.2011.59 2-s2.0-79955020031 21442146 PMC5720057 15 Isaacson P. G. Mucosa-Associated Lymphoid Tissue Lymphoma Seminars in Hematology 1999 April 36 2 139 147 10319382 …

Reversing Autoimmunity Combination of Rituximab and Intravenous Immunoglobulin.

In this concept paper, the authors present a unique and novel protocol to treat autoimmune diseases that may have the potential to reverse autoimmunity. It uses a combination of B cell depletion therapy (BDT), specifically rituximab (RTX) and intravenous immunoglobulin (IVIg), based on a specifically designed protocol (Ahmed Protocol). Twelve infusions of RTX are given in 6-14 months. Once the CD20+ B cells are depleted from the peripheral blood, IVIg is given monthly until B cells repopulation occurs. Six additional cycles are given to end the protocol. During the stages of B cell depletion,

ЦАРТ клетки в качестве новой терапевтической стратегии для множественных склерозов и других нейроиммунных расстройств.

В качестве трансформационного подхода к лечению множественных склерозов (MS) и других нейроиммунных расстройств, таких, как расстройства спектра нейромиелита (NMOSD), миелин олигодендроцита гликпротеина, связанных с антителами (MOGAD), и миастения гравис (MG), наряду с рядом других редких нейроиммунологических заболеваний, которые в настоящее время оцениваются в исследованиях, посвященных сочувствию или ранней фазе. Отчасти эти условия обусловлены автоматически реактивными клетками B, вызывающими хроническое воспаление и прогрессивное повреждение тканей. Несмотря на то, что нынешняя иммунодуля

Relapsed Multiple Myeloma Presenting as Intracranial Plasmacytoma and Malignant Pericardial Effusion following Recent Allogeneic Stem Cell Transplantation.

Although rare, both central nervous system and pericardial involvement of myeloma have been well described in the literature. Their simultaneous occurrence in relapsed disease, however, has not been previously reported. This case describes a 54-year-old female who was treated for high-risk multiple myeloma with multiregimen chemotherapy and allogeneic hematopoietic stem cell transplantation. Four months after transplant, she was found to have relapsed disease manifesting as an extraosseous, intracranial plasmacytoma and simultaneous malignant pericardial effusion. Her disease characteristics,

Clinicopathological Features and Prognostic Differences Between Primary and Relapsed or Refractory Extramedullary Multiple Myeloma.

PURPOSE: This study aims to explore the clinicopathological features related to extramedullary multiple myeloma (EMM) occurrence and evaluate prognosis differences between patients with primary EMM (PEMM) and those with relapsed or refractory EMM (R/REMM). PATIENTS AND METHODS: The laboratory test results, clinical, histopathological and immunophenotypic characteristics, cytogenetic changes and prognosis of 203 patients with MM were obtained. The patients were categorized into EMM+ and EMM- groups. Patients in the EMM+ group were further categorized into the PEMM and R/REMM groups based on th

Эффективное использование BCMA-нацеливаемого би-специализированного T антитела в реанимации LRP4+ yasthenia gravis.

Myastenia gravis (MG) — аутоиммунное заболевание, вызванное антителом, которое воздействует на нейромышечное соединение. Рефракторный MG, особенно в случаях, связанных с редким антинизкоплотным белком, связанным с рецептором липопротеина 4 (LRP4) Антитела сталкиваются со значительными трудностями в лечении. Teclistamab, биконкретный антитело, нацеленное на антигены созревания клеток B (BCMA) и CD3, перенаправляет Т клетки на плазменные клетки и утверждается для лечения нескольких миелом. Растущий интерес связан с использованием методов лечения на основе Т клеток в клеточных заболеваниях с ауто