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Budd-Chiari syndrome in myeloproliferative neoplasms:A review of literature

World Journal of Clinical Oncology 2023年第3期14卷 99-116页

作者： Mihnea-Alexandru Găman Matei-Alexandru Cozma Muhammad Romail Manan Bahadar S Srichawla Arkadeep Dhali sajjad Ali ahmed Nahian Andrew C Elton L V Simhachalam Kutikuppala Richard Christian Suteja Sebastian Diebel Amelia Maria Găman Camelia Cristina Diaconu Faculty of Medicine “Carol Davila”University of Medicine and PharmacyBucharest 050474Romania Department of Hematology Center of Hematology and Bone Marrow TransplantationFundeni Clinical InstituteBucharest 022328Romania Services Institute of Medical Sciences Lahore 54660Pakistan Department of Neurology University of Massachusetts Chan Medical SchoolWorcesterMassachusetts 01655United States Department of Internal Medicine Nottingham University Hospitals NHS TrustNottingham NG72UHUnited Kingdom Department of Medicine Ziauddin UniversityKarachi 75600Pakistan California Baptist University-Lake Erie College of Osteopathic Medicine RiversideCA 92504United States University of Minnesota Medical School MinneapolisMN 55455United States Department of General Surgery Dr NTR University of Health SciencesAndhra PradeshVijayawada 520008India Department of Undergraduate Medicine Faculty of MedicineUdayana University DenpasarBali 80232Indonesia Department of Family Medicine Northern Ontario School of Medicine TimminsOntario 91762Canada Department of Pathophysiology University of Medicine and Pharmacy of CraiovaRomania&Clinic of HematologyFilantropia City HospitalCraiova 200143Romania Department of Internal Medicine Faculty of Medicine“Carol Davila”University of Medicine and PharmacyRomania&Internal Medicine ClinicClinical Emergency Hospital of BucharestBucharest 105402Romania

Myeloproliferative neoplasms(MPNs)are defined as clonal disorders of the hematopoietic stem cell in which an exaggerated production of terminally differentiated myeloid cells ***,Philadelphia-negative MPNs,i.e.,polycythemia vera,essential thrombocythemia and primary myelofibrosis,exhibit a propensity towards the development of thrombotic complications that can occur in unusual sites,e.g.,portal,splanchnic or hepatic veins,the placenta or cerebral *** pathogenesis of thrombotic events in MPNs is complex and requires an intricate mechanism involving endothelial injury,stasis,elevated leukocyte adhesion,integrins,neutrophil extracellular traps,somatic mutations(e.g.,the V617F point mutation in the JAK2 gene),microparticles,circulating endothelial cells,and other factors,to name a ***,we review the available data on Budd-Chiari syndrome in Philadelphia-negative MPNs,with a particular focus on its epidemiology,pathogenesis,histopathology,risk factors,classification,clinical presentation,diagnosis,and management.

关键词： Myeloproliferative neoplasms Budd-Chiari syndrome Thrombosis Polycythemia vera Essential thrombocythemia Primary myelofibrosis

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