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Copyright ©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Oncol. Mar 24, 2023; 14(3): 99-116
Published online Mar 24, 2023. doi: 10.5306/wjco.v14.i3.99
Budd-Chiari syndrome in myeloproliferative neoplasms: A review of literature
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
Mihnea-Alexandru Găman, Matei-Alexandru Cozma, Faculty of Medicine, “Carol Davila” University of Medicine and Pharmacy, Bucharest 050474, Romania
Mihnea-Alexandru Găman, Department of Hematology, Center of Hematology and Bone Marrow Transplantation, Fundeni Clinical Institute, Bucharest 022328, Romania
Muhammad Romail Manan, Services Institute of Medical Sciences, Lahore 54660, Pakistan
Bahadar S Srichawla, Department of Neurology, University of Massachusetts Chan Medical School, Worcester, Massachusetts 01655, United States
Arkadeep Dhali, Department of Internal Medicine, Nottingham University Hospitals NHS Trust, Nottingham NG7 2UH, United Kingdom
Sajjad Ali, Department of Medicine, Ziauddin University, Karachi 75600, Pakistan
Ahmed Nahian, California Baptist University-Lake Erie College of Osteopathic Medicine, Riverside, CA 92504, United States
Andrew C Elton, University of Minnesota Medical School, Minneapolis, MN 55455, United States
L V Simhachalam Kutikuppala, Department of General Surgery, Dr NTR University of Health Sciences, Andhra Pradesh, Vijayawada 520008, India
Richard Christian Suteja, Department of Undergraduate Medicine, Faculty of Medicine, Udayana University Denpasar, Bali 80232, Indonesia
Sebastian Diebel, Department of Family Medicine, Northern Ontario School of Medicine Timmins, Ontario 91762, Canada
Amelia Maria Găman, Department of Pathophysiology, University of Medicine and Pharmacy of Craiova, Romania & Clinic of Hematology, Filantropia City Hospital, Craiova 200143, Romania
Camelia Cristina Diaconu, Department of Internal Medicine, Faculty of Medicine, “Carol Davila” University of Medicine and Pharmacy, Romania & Internal Medicine Clinic, Clinical Emergency Hospital of Bucharest, Bucharest 105402, Romania
Author contributions: All authors contributed to writing the original draft; Găman MA, Găman AM and Diaconu CC contributed to writing, review and editing, and methodology; Găman AM and Diaconu CC contributed equally as senior/last authors; all authors read and approved the final version of this paper.
Conflict-of-interest statement: The authors have no conflicts of interest to declare.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Mihnea-Alexandru Găman, Doctor, MD, PhD, Research Fellow, Researcher, Faculty of Medicine, “Carol Davila” University of Medicine and Pharmacy, 8 Eroii Sanitari Blvd, Bucharest 050474, Romania. mihneagaman@yahoo.com
Received: November 20, 2022
Peer-review started: November 20, 2022
First decision: January 3, 2023
Revised: February 10, 2023
Accepted: March 1, 2023
Article in press: March 1, 2023
Published online: March 24, 2023
Processing time: 119 Days and 6.5 Hours
Abstract

Myeloproliferative neoplasms (MPNs) are defined as clonal disorders of the hematopoietic stem cell in which an exaggerated production of terminally differentiated myeloid cells occurs. Classical, 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 sinuses. The 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 few. Herein, 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.

Keywords: Myeloproliferative neoplasms; Budd-Chiari syndrome; Thrombosis; Polycythemia vera; Essential thrombocythemia; Primary myelofibrosis

Core Tip: Myeloproliferative neoplasms (MPNs) are defined as clonal disorders of the hematopoietic stem cell in which an exaggerated production of terminally differentiated myeloid cells occurs. MPNs are characterized by a propensity towards the development of thrombotic complications, including Budd-Chiari syndrome (BCS). Herein, we review the available data on BCS in MPNs, with a particular focus on its epidemiology, pathogenesis, histopathology, risk factors, classification, clinical presentation, diagnosis, and management.