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Systemic mastocytosis: current status and challenges in 2024.

AbstractSystemic mastocytosis (SM) is a rare disease and has had significant discoveries in its biology, prognostication, and management in the past 2 decades. The latest update of the World Health Organization classification and the new International Consensus Classification are current standards in the diagnosis and prognostication of SM. In clinical practice, SM can be divided into 2 main categories: nonadvanced SM (nonAdvSM) and advanced SM (AdvSM). The integration of clinical signs and symptoms as well as bone marrow morphologic, immunophenotypic, and molecular results is required to diag

Epidemiology, Clinical Data, and Management of Aseptic Abscess Syndrome: Review of Published Cases Outside France.

Aseptic abscess syndrome is a clinical entity that is being increasingly documented. Unfortunately, apart from the French registry, there are no other studies presenting collective data. In this review, we sought to analyze clinical and laboratory data from case reports published from the rest of the world. A total of 107 articles were found through our literature search in PubMed, Scopus, and Google, which contained 108 patients who met our eligibility criteria, including pediatric cases. The mean age at diagnosis was 39.1 years, and 54.6% of the patients were female. Cases were found affecti

Primary tumours of the calcaneus.

The calcaneus is a rare location for the occurrence and development of primary tumour types. Clinicians are unfamiliar with calcaneal tumour types, which may result in a delay in diagnosis or a missed diagnosis, resulting in unnecessary morbidity and amputation. Heel pain and localized swelling of the ankle are the most common symptoms. X-ray is the first choice for a tentative diagnosis of a calcaneal tumour. The final diagnosis depends on a histological examination. The treatment of calcaneal tumour types varies depending on the Enneking system. The majority of patients with benign tumours h

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… Histopathologic examination of the lesion reveals cavernous cavities of different dimensions containing fibroblasts, histiocytes and osteoclastic giant cells without any mitosis or atypia ( 19 , 21 , 22 ). …

Hematopoietic Tumors

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Искусственный интеллект, видоизменяющий парадигму гематологического диагноза и лечения злокачественных заболеваний: от статической оценки до динамического управления точностью.

Искусственный интеллект (ИИ) все чаще изучается в качестве инструмента содействия более точному и динамичному управлению диагностикой и лечением гематологов. В отличие от предыдущих обзоров, в которых основное внимание уделялось отдельным типам заболеваний или изолированным технологическим путям, в настоящем документе содержится всеобъемлющий обзор текущих прикладных программ АИШ и последних достижений в области диагностики, классификации, оценки прогнозов и принятия решений по лечению лейкемии, лимфомы, множественной миэломы и миэлодиспластических синдромов. Она охватывает ключевые технически

Toward a more patient-centered drug development process in clinical trials for patients with myelodysplastic syndromes/neoplasms (MDS): Practical considerations from the International Consortium for MDS (icMDS).

Notable treatment advances have been made in recent years for patients with myelodysplastic syndromes/neoplasms (MDS), and several new drugs are under development. For example, the emerging availability of oral MDS therapies holds the promise of improving patients' health-related quality of life (HRQoL). Within this rapidly evolving landscape, the inclusion of HRQoL and other patient-reported outcomes (PROs) is critical to inform the benefit/risk assessment of new therapies or to assess whether patients live longer and better, for what will likely remain a largely incurable disease. We provide

Exosomal delivery of TRAIL and miR‑335 for the treatment of hepatocellular carcinoma (Review).

Liver cancer is the sixth most prevalent type of cancer worldwide and accounts for the third most frequent cause of cancer‑associated mortality. Conventional anticancer drugs display limited efficacy owing to their short half‑life, poor solubility and inefficient drug delivery. Despite advancements being made in drug discovery and development for the treatment of hepatocellular carcinoma (HCC), drug inefficacy and drug continue to pose significant obstacles to effective treatment. Therefore, it is imperative that novel treatment strategies be developed with the aim of developing anticancer tre

Heterogeneity in precision oncology.

Precision oncology is a rapidly evolving concept that holds great promise in cancer treatment. However, a cancer complexity attributed to genomic and acquired tumour heterogeneity limits treatment effectiveness and increases toxicity. These limitations refer to both systemic therapies and radiotherapy, which are two mainstays of non-invasive cancer treatment. By understanding cancer heterogeneity and utilising advanced tools to personalise treatment strategies, precision oncology has the potential to revolutionise cancer care. In this article, we review the current status of precision oncology

Биология и болезни крыс

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Development Trends of Janus Kinase Inhibitors (JAKi) Over Three Decades: From Common to Rare Diseases.

Janus kinase inhibitors (JAKi) have undergone three decades of research and development, demonstrating substantial therapeutic efficacy and broad clinical applicability in autoimmune diseases, inflammatory disorders, hematopoietic malignancies, and a spectrum of rare diseases. However, their research and development are constrained by three key challenges: the difficulty in target selection and safety concerns arising from their effects on cellular signaling pathways, as well as the challenges in formulating optimal dosing regimens due to interindividual variability in pharmacokinetics. In thi

Advances in Leukemia detection and classification: A Systematic review of AI and image processing techniques.

BACKGROUND: Leukemia, a heterogeneous group of blood cancers, poses significant challenges to global health due to its complexity, diverse risk factors, and variable outcomes. Accurate and early diagnosis is critical but remains a significant hurdle, particularly in low-resource settings. Recent advancements in artificial intelligence (AI) and image processing offer transformative solutions to improve leukemia detection and classification, addressing limitations in traditional diagnostic methods. METHODS: This study systematically reviewed over 25,000 scientific articles sourced from Scopus,

Systemic complications of rheumatoid arthritis: Focus on pathogenesis and treatment.

As a systemic autoimmune disease, rheumatoid arthritis (RA) usually causes damage not only to joints, but also to other tissues and organs including the heart, kidneys, lungs, digestive system, eyes, skin, and nervous system. Excessive complications are closely related to the prognosis of RA patients and even lead to increased mortality. This article summarizes the serious complications of RA, focusing on its incidence, pathogenesis, clinical features, and treatment methods, aiming to provide a reference for clinicians to better manage the complications of RA.

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… Early diagnosis and effective treatment of pericarditis will significantly improve the prognosis of RA patients. 2.1.2 Myocarditis Myocarditis is the result of persistent inflammation in the myocardium and is histologically characterized by cellular infiltration composed of lymphocytes, histiocytes …

Understanding DMG: current treatment options and prospective solutions using nanoparticles.

Diffuse Midline Gliomas (DMGs), which include the subset formerly known as Diffuse Intrinsic Pontine Glioma (DIPG), are associated with an extremely poor prognosis and a five-year survival rate below 2-3%. The devastating prognosis stems from an inherently invasive nature, a deep, anatomically restricted location, rapid cellular drug efflux mediated by transporters such as P-glycoprotein (ABCB1) and breast cancer resistance protein (BCRP/ABCG2), and an immunologically "cold" microenvironment. Coupled with limited Blood-Brain Barrier (BBB) permeability, these biological factors render conventio

The progress of novel strategies on immune-based therapy in relapsed or refractory diffuse large B-cell lymphoma.

Diffuse large B-cell lymphoma (DLBCL) can be cured with standard front-line immunochemotherapy, whereas nearly 30-40% of patients experience refractory or relapse. For several decades, the standard treatment strategy for fit relapsed/refractory (R/R) DLBCL patients has been high-dose chemotherapy followed by autologous hematopoietic stem cell transplant (auto-SCT). However, the patients who failed in salvage treatment or those ineligible for subsequent auto-SCT have dismal outcomes. Several immune-based therapies have been developed, including monoclonal antibodies, antibody-drug conjugates, b

Burden, Outcome, and Comorbidities of Extrahepatic Manifestations in Hepatitis B Virus Infections.

Hepatitis B virus (HBV) infections affect approximately 296 million people around the world, and the prevalence of any past or present HBV infection during the years 2015-2018 was as high as 4.3%. Acute HBV infection often presents with nonspecific symptoms and is usually self-limited, but 5% of patients can have persistent infections leading to chronic HBV infection and the risk of turning into chronic HBV infection is significantly higher in babies with vertical transmission (95%). Patients with chronic HBV infection are usually asymptomatic, but 15 to 40% of chronic HBV carriers develop cir

Acute Generalized Exanthematous Pustulosis: Clinical Features, Differential Diagnosis, and Management.

Acute generalized exanthematous pustulosis (AGEP) is a rare, acute, severe cutaneous adverse reaction mainly attributed to drugs, although other triggers, including infections, vaccinations, ingestion of various substances, and spider bites, have also been described. AGEP is characterized by the development of edema and erythema followed by the eruption of multiple punctate, non-follicular, sterile pustules and subsequent desquamation. AGEP typically has a rapid onset and prompt resolution within a few weeks. The differential diagnoses for AGEP are broad and include infectious, inflammatory, a

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… Fig. 2 Representative HE histology of AGEP depicting ( a ) a subcorneal neutrophilic pustule, ( b ) vacuolization of basal keratinocytes in the adjacent epidermis, accompanied by ( c ) a mixed infiltrate in the upper dermis consisting of lymphocytes, histiocytes, neutrophils and admixed eosinophils. …

The 8p11 myeloproliferative syndrome: Genotypic and phenotypic classification and targeted therapy.

EMS(8p11 myeloproliferative syndrome, EMS) is an aggressive hematological neoplasm with/without eosinophilia caused by a rearrangement of the FGFR1 gene at 8p11-12. It was found that all cases carry chromosome abnormalities at the molecular level, not only the previously reported chromosome translocation and insertion but also a chromosome inversion. These abnormalities produced 17 FGFR1 fusion genes, of which the most common partner genes are ZNF198 on 13q11-12 and BCR of 22q11.2. The clinical manifestations can develop into AML (acute myeloid leukemia), T-LBL (T-cell lymphoblastic lymphoma),

Inflammatory stromal and T cells mediate human bone marrow niche remodeling in clonal hematopoiesis and myelodysplasia.

Somatic mutations in hematopoietic stem/progenitor cells (HSPCs) can lead to clonal hematopoiesis of indeterminate potential (CHIP) and progression to myelodysplastic syndromes (MDS). Using single-cell and anatomical profiling of a large cohort of human bone marrow (BM), we show that the HSPC BM niche in CHIP and MDS is undergoing inflammatory remodeling. This includes loss of CXCL12⁺ adipogenic stromal cells and the emergence of a distinct population of inflammatory mesenchymal stromal cells (iMSCs), which arise in CHIP and become more prevalent in MDS. Functional studies in primary BM HSPC-M

An overview of arsenic trioxide-involved combined treatment algorithms for leukemia: basic concepts and clinical implications.

Arsenic trioxide is a first-line treatment drug for acute promyelocytic leukemia, which is also effective for other kinds of leukemia. Its side effects, however, limit its clinical application, especially for patients with complex leukemia symptoms. Combination therapy can effectively alleviate these problems. This review summarizes the research progress on the combination of arsenic trioxide with anticancer drugs, vitamin and vitamin analogs, plant products, and other kinds of drugs in the treatment of leukemia. Additionally, the new progress in arsenic trioxide-induced cardiotoxicity was sum

Molecular Mechanisms Leading from Periodontal Disease to Cancer.

Periodontitis is prevalent in half of the adult population and raises critical health concerns as it has been recently associated with an increased risk of cancer. While information about the topic remains somewhat scarce, a deeper understanding of the underlying mechanistic pathways promoting neoplasia in periodontitis patients is of fundamental importance. This manuscript presents the literature as well as a panel of tables and figures on the molecular mechanisms of Porphyromonas gingivalis and Fusobacterium nucleatum, two main oral pathogens in periodontitis pathology, involved in instigati

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… In adult human tissues, specific staining for RANKL could be detected in bone (osteoblasts), lungs (alveolar cells), sweat glands (epithelial cells), tonsils (crypt epithelium), lymph nodes (lymphocytes and histiocytes) as well as in macrophages scattered within various tissue types [ 97 ]. …