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IgM Myeloma: Всеобъемлющий обзор и практический подход к хемапаевому управлению.

IgM mieloma — редкий подтип множественной миэломы (MM). 0.5% из всех его дел. Она характеризуется нерегулируемым распространением плазменных клеток ИгМ в костном мозге. Фундаментальный патогенез предполагает регулирование изотипной рекомбинации переключателей, что приводит к различным транслокациям хромосом, таким, как 11q13 и 4p16. Обычно у пациентов возникают симптомы гипервизорного синдрома, инфильтрации костного мозга и органомегалия. Диагностика включает в себя клиническую оценку, лабораторные тесты (электропорез, биопсия костного мозга, цитогенетика, иммунохимия) и съемку. Варианты лечен

Prognosis of concurrent renal impairment at diagnosis of multiple myeloma: a systematic review.

BackgroundMultiple myeloma is a malignant tumour of the blood in which abnormal proliferation of plasma cells leads to bone destruction, renal impairment, anaemia, and hypercalcaemia. Renal impairment caused by multiple myeloma is a common and serious condition; however, the prognosis of multiple myeloma at the time of diagnosis remains unclear.MethodWe conducted searches for literature in PubMed, Web of Science, Cochrane, Embase, CNKI, Wanfang, and VIP databases up to 30 April 2023. Progression-free survival and overall survival with and without renal impairment at the time of multiple myelom

Autoimmune Rheumatic Diseases and Vascular Function: The Concept of Autoimmune Atherosclerosis.

Autoimmune rheumatic diseases (AIRDs) with unknown etiology are increasing in incidence and prevalence. Up to 5% of the population is affected. AIRDs include rheumatoid arthritis, system lupus erythematosus, systemic sclerosis, and Sjögren's syndrome. In patients with autoimmune diseases, the immune system attacks structures of its own body, leading to widespread tissue and organ damage, which, in turn, is associated with increased morbidity and mortality. One third of the mortality associated with autoimmune diseases is due to cardiovascular diseases. Atherosclerosis is considered the main un

Pentoxifylline and Tocopherol for the Management of Medication-Related Osteonecrosis of the Jaw (MRONJ): A Retrospective Clinical Audit.

Background/Objectives: Medication-related osteonecrosis of the jaw (MRONJ) is a challenging complication in patients receiving antiresorptive therapy. Management strategies range from conservative pharmacological approaches to extensive surgical resection of necrotic bone. This clinical audit retrospectively evaluated the clinical outcomes of patients undergoing sequestrectomy for MRONJ, comparing those treated with antibiotics alone with those receiving antibiotics in combination with the pentoxifylline-tocopherol (PENTO) protocol. The PENTO protocol was introduced at our institution in 2021

Pathogenesis and Treatment of Myeloma-Related Bone Disease.

Multiple myeloma is a hematologic malignancy of plasma cells that causes bone-destructive lesions and associated skeletal-related events (SREs). The pathogenesis of myeloma-related bone disease (MBD) is the imbalance of the bone-remodeling process, which results from osteoclast activation, osteoblast suppression, and the immunosuppressed bone marrow microenvironment. Many important signaling cascades, including the RANKL/RANK/OPG axis, Notch signaling, the Wnt/β-Catenin signaling pathways, and signaling molecules, such as DKK-1, sclerostin, osteopontin, activin A, chemokines, and interleukins

Incidence of malignancies after lung transplantation and their effect on the outcome. 26 years' experience.

BackgroundMalignancy is a significant, life-limiting complication after lung transplantation (LuTx) and the second common long-term cause of death. We aimed to investigate its incidence and effect on the outcome.MethodsThis is a retrospective observational study. Between 1996 and 2022, n = 627 lung transplantations (LuTx) were performed in our department. We used our institutional database to identify recipients with malignancies after LuTx and examined the malignancies' incidence and mortality.ResultsN = 59 malignancies occurred in n = 55 (8.8%) LuTx recipients. The post-LTx malignancies inci

C-Reactive Protein: Pathophysiology, Diagnosis, False Test Results and a Novel Diagnostic Algorithm for Clinicians.

The current literature provides a body of evidence on C-Reactive Protein (CRP) and its potential role in inflammation. However, most pieces of evidence are sparse and controversial. This critical state-of-the-art monography provides all the crucial data on the potential biochemical properties of the protein, along with further evidence on its potential pathobiology, both for its pentameric and monomeric forms, including information for its ligands as well as the possible function of autoantibodies against the protein. Furthermore, the current evidence on its potential utility as a biomarker of

Cardiovascular toxicity from therapies for light chain amyloidosis.

Amyloid light-chain (AL) amyloidosis is a hematological disorder characterized by abnormal proliferation of a plasma cell clone producing monoclonal free light chains that misfold and aggregate into insoluble fibrils in various tissues. Cardiac involvement is a common feature leading to restrictive cardiomyopathy and poor prognosis. Current first-line treatments aim at achieving hematological response by targeting the plasma cell clones, and these have been adapted from multiple myeloma therapy. Patients with AL amyloidosis often exhibit multiorgan involvement, making them susceptible to cance

MPO-ANCA-Associated Hypertrophic Pachymeningitis Mimicking IgG4-Related Disease: A Case Report and Literature Review.

Hypertrophic pachymeningitis (HP) is a rare and chronic clinical disease characterized by thickening of the dura mater, leading to persistent headache, cranial neuropathy, seizures, and other neurological symptoms. Immune-mediated causes, particularly antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and IgG4-related disease (IgG4-RD), are among the most common etiologies. We report a case of a 54-year-old female with recurrent headache, blepharoptosis, hearing loss, and markedly elevated inflammatory markers. Blood tests, and serum levels of IgG4 were within normal ranges. Cont

Preclinical Evaluation of a Lentiviral Vector for Huntingtin Silencing.

Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder resulting from a polyglutamine expansion in the huntingtin (HTT) protein. There is currently no cure for this disease, but recent studies suggest that RNAi to downregulate the expression of both normal and mutant HTT is a promising therapeutic approach. We previously developed a small hairpin RNA (shRNA), vectorized in an HIV-1-derived lentiviral vector (LV), that reduced pathology in an HD rodent model. Here, we modified this vector for preclinical development by using a tat-independent third-generation LV (pCCL) ba

Inhibition of EIF2α Dephosphorylation Decreases Cell Viability and Synergizes with Standard-of-Care Chemotherapeutics in Head and Neck Squamous Cell Carcinoma.

Drug resistance is a common cause of therapy failure in head and neck squamous cell carcinoma (HNSCC). One approach to tackling it is by targeting fundamental cellular processes, such as translation. The eukaryotic translation initiation factor 2α (EIF2α) is a key player in canonical translation initiation and integrates diverse stress signals; when phosphorylated, it curbs global protein synthesis. This study evaluates EIF2α expression and phosphorylation in HNSCC. A small-molecule inhibitor of EIF2α dephosphorylation, salubrinal, was tested in vitro, followed by viability assays, flow cytome

ROS 2020 Abstract

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Haematological malignancies and acute kidney injury requiring nephrology consultation: challenging the worst of the worst.

BackgroundAcute kidney injury (AKI) often complicates the course of haematological malignancies (HMs) and confers a worse prognosis. The majority of these patients are managed by the attending physician, yet, a small group, mostly coincident with the worst presentation and outcomes, requires nephrology consultation, challenging the clinician with ethical issues regarding the decision to initiate or forgo renal support therapy. The purpose of this work is to identify the prognostic determinants for in-hospital mortality in this population.MethodsA retrospective, observational chart review was u

A Case Study of a Rare Disease (Fructosemia) Diagnosed in a Patient with Abdominal Pain.

Hereditary fructose intolerance is a rare genetic disorder that is inherited in an autosomal recessive manner, with mutations sometimes occurring spontaneously. Consuming fructose triggers biochemical abnormalities, disrupting liver processes like glycogenolysis and gluconeogenesis. Recent studies have revealed elevated intrahepatic fat levels in affected individuals. Symptoms include aversion to fructose-containing foods, hypoglycemia, liver and kidney dysfunction, and growth delays, with severe cases leading to liver enlargement, fatty liver disease, kidney failure, and life-threatening hypo

Аберрантная дополняющая активация связана со структурным повреждением мозга при множественном склерозе.

Уровни активированных дополнительных белков в CSF увеличиваются у людей с множественным склерозом (MS) и связаны с остротой клинических заболеваний. В этом исследовании мы определили, следует ли дополнять профили активации количественными показателями МРТ и жидкими биомаркерами, указывающими на активность и прогресс в области заболеваний.МЕТОДЫ: Дополняющие компоненты и продукты активации (Factor H и I, C1q, C3, C4, C5, Ba, Bb, C3a, C4a, C5a и sC5b-9) и жидкие биомаркеры (белковая цепочка накаливания, глиальный фибриллярный кислотный белок [GFAP], CXCL-13, CXCL-9, и IL-12b), были количественно

Nucleotide Metabolism in Health and Disease.

Nucleotide metabolism is a fundamental biochemical process, providing the building blocks for DNA and RNA synthesis while driving critical cellular functions like energy transfer, signal transduction, and coenzyme synthesis. The precise regulation of nucleotide de novo synthesis, salvage, and degradation pathways is imperative for maintaining genomic stability and tissue homeostasis. Aberrations in these networks are increasingly recognized as primary drivers in various pathologies; notably, cancer cells exploit metabolic-functional reprogramming to enable rapid proliferation and therapeutic r

CARBOMETASPINE: protocol for a multicenter, prospective, randomized controlled trial of carbonfiber spinal fixation in metastatic disease.

BACKGROUND: Spinal metastases frequently cause neurological compromise and mechanical instability. Titanium fixation limits postoperative imaging and hinders stereotactic body radiotherapy (SBRT) planning because of artefacts. METHODS: CARBOMETASPINE is a multicentre, prospective, randomised controlled trial. A total of 226 adults with unstable or epidurally infiltrating spinal metastases will be randomised 1 : 1 : 1 to: (I) carbonfibre/PEEK fixation + postoperative SBRT (5 × 5 Gy); (II) titanium fixation + postoperative SBRT; (III) preoperative SBRT + titanium fixation. The primary endpoint i

Real-World Outcomes of Patients with Monoclonal Gammopathy of Renal Significance.

BACKGROUND: Monoclonal gammopathy of renal significance (MGRS) is a rare group of renal disorders caused by monoclonal immunoglobulins from non-malignant B-cell or plasma cell clones, with distinct amyloid (MGRS-A) and non-amyloid (MGRS-NA) subtypes. Although these are often grouped together, whether they share similar clinical phenotypes and outcomes is uncertain. We therefore examined similarities and differences between these subtypes in a contemporary real-world cohort. METHODS: We performed a single-centre retrospective evaluation of 17 biopsy-confirmed MGRS patients (10 MGRS-A, 7 MGRS-N

Extramedullary disease is associated with severe toxicities following B-cell maturation antigen CAR T-cell therapy in multiple myeloma.

Extramedullary disease (EMD) in multiple myeloma (MM) is associated with poor outcomes following B-cell maturation antigen (BCMA)-targeted chimeric antigen receptor (CAR) T therapy, yet its impact on treatment-related toxicity remains unclear. This study evaluates the impact of active EMD on toxicity, efficacy, and survival in patients with MM treated with idecabtagene vicleucel (ide-cel) or ciltacabtagene autoleucel (cilta-cel). We conducted a retrospective cohort study of all patients with MM who received ide-cel (N=32) or cilta-cel (N=76) as standard-of-care therapy at our institution from

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… There was also a trend toward more prolonged peak International Normalized Ratio (INR) values (1.30 vs. 1.20; P =0.067). … Box plots depicting levels of (A) C-reactive protein (CRP), (B) D-dimer, (C) ferritin, (D) interleukin (IL)-6, (E) lactate dehydrogenase (LDH), (F) International Normalized Ratio (INR), and (G) fibrinogen (FBG) at various time points relative to chimeric antigen receptor (CAR) T infusion, stratified …

Изучение связи между 91 циркуляцией воспламенительных протеинов и нейродегенеративными заболеваниями: двухнаправленный анализ менделей и биоинформатики.

Циркуляция подстрекательских белков играет важную роль в патогенезе нейродегенеративных заболеваний (НДД). Однако точная причинно-следственная связь и механизмы, лежащие в основе их взаимодействия, по-прежнему остаются неясными. МЕТОДЫ: В каталоге GWAS были получены данные по 91 циркуляционному подстрекательскому протеину в рамках исследования ассоциации геномов (GWAS). Кроме того, данные GWAS о болезни Паркинсона (PD), болезни Альцгеймера (AD), амиотропическом боковом склерозе (ALS), множественном склерозе (MS) и ишемическом инсульте (IS) были получены в рамках проекта IEU Open GWAS. Для анал