Myeloproliferative neoplasms can present early in life and may present a diagnostic challenge. Very few studies have focused on the diagnosis, prognosis, and therapy for pediatric myeloproliferative neoplasms. This article focuses on chronic myeloid leukemia, essential thrombocythemia, polycythemia vera, and primary myelofibrosis in children.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.cll.2021.04.010 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Design and fabrication of novel microfluidic-based droplets for drug screening on a chronic myeloid leukemia cell line.
PLoS One
January 2025
Department of Hematology and Blood Banking, Faculty of Allied Medicine, Iran University of Medical Science, Tehran, Iran.
Background: The challenges associated with traditional drug screening, such as high costs and long screening times, have led to an increase in the use of single-cell isolation technologies. Small sample volumes are required for high-throughput, cell-based assays to reduce assay costs and enable rapid sample processing. Using microfluidic chips, single-cell analysis can be conducted more effectively, requiring fewer reagents and maintaining biocompatibility.
View Article and Find Full Text PDFLactate accumulation promotes immunosuppression and fibrotic transformation of bone marrow microenvironment in myelofibrosis.
J Transl Med
January 2025
Department of Biomedical and Biotechnological Sciences, Division of Medical Biochemistry, University of Catania, Catania, Italy.
Background: Clonal myeloproliferation and fibrotic transformation of the bone marrow (BM) are the pathogenetic events most commonly occurring in myelofibrosis (MF). There is great evidence indicating that tumor microenvironment is characterized by high lactate levels, acting not only as an energetic source, but also as a signaling molecule.
Methods: To test the involvement of lactate in MF milieu transformation, we measured its levels in MF patients' sera, eventually finding a massive accumulation of this metabolite, which we showed to promote the expansion of immunosuppressive subsets.
Fedratinib for the treatment of myelofibrosis: a critical appraisal of clinical trial and "real-world" data.
Blood Cancer J
January 2025
School of Medicine, Faculty of Medicine and Health Sciences, Tel Aviv University, Tel Aviv, Israel.
Fedratinib is a predominantly JAK2 inhibitor that has shown efficacy in untreated and ruxolitinib-exposed patients with myelofibrosis (MF). Based on randomized clinical trial data, it is approved for use in patients with International Prognostic Scoring System (IPSS) or Dynamic International Prognostic Scoring System (DIPSS) intermediate-2 or high-risk disease and is distinguished from ruxolitinib in that it can be administered without dose reduction in patients with thrombocytopenia, to a platelet count above 50,000/µL. In these trials, fedratinib achieved significant spleen volume reduction in ~30-45% of patients and improvement in total symptom scores in 35-40% with good tolerability.
View Article and Find Full Text PDFEndothelial Damage in JAK2V617F Myeloproliferative Neoplasms with Splanchnic Vein Thrombosis.
Thromb Haemost
January 2025
Hemostasis and Erythropathology Laboratory, Hematopathology, Pathology Department, Centre de Diagnòstic Biomèdic (CDB), Hospital Clínic de Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
Background: V617F-mutated myeloproliferative neoplasms (MPN) exhibit abnormal proliferation of bone marrow progenitors and increased risk of thrombosis, specifically in splanchnic veins (SVT). The contribution of the endothelium to the development of the prothrombotic phenotype was explored.
Material And Methods: Plasma and serum samples from V617F MPN patients with (n=26) or without (n=7) thrombotic debut and different treatments, were obtained (n=33).
Background: Platelets are correlated with myeloid leukemia (ML), but to date, there have been no studies confirming the causal relationship between them.
Methods: Platelet count (PLT), mean platelet volume (MPV), plateletcrit (PCT), and platelet distribution width (PDW) data were obtained from the GWAS catalog database as exposure factors. Acute myeloid leukemia (AML) and chronic myeloid leukemia (CML) data were obtained from the FinnGen database as outcome indicators.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!