Volume : 10, Issue : 08, August – 2023
Title:
15.PORTAL VEIN CAVERNOUS TRANSFORMATION IN A PATIENT WITH ESSENTIAL THROMBOCYTHEMIA: A RARE AND INTERESTING PRESENTATION
Authors :
Muhammad Hamza Mushtaq, Malik Hasnat Ul Hassan Khan, Muhammad Tabish Ikram, Sarah Nisar, Asad Rahman, Syed Aftab Ahmad
Abstract :
Essential thrombocythemia is a myeloproliferative disorder characterized by an elevated platelet count, with symptoms such as vasospasm and a predisposition to bleeding and microvascular complications. While rare, one complication that can arise from chronic portal vein thrombosis is the cavernous transformation of the portal vein. In this case report, we explore a unique presentation of cavernous transformation of the portal vein (CTPV) in a patient diagnosed with essential thrombocythemia (ETC). This case report gives importance to considering myeloproliferative disorders like ETC as a potential cause of chronic portal vein thrombosis leading to the CTPV.This case report describes a 26-year-old male patient diagnosed with cavernous transformation of the portal vein (CTPV) during a workup for hematemesis and melena. The patient was found to have severe anemia, thrombocytosis, splenomegaly, and a positive JAK2 mutation, consistent with a diagnosis of essential thrombocythemia. Although thrombotic events in essential thrombocythemia patients are common, portal vein thrombosis and CTPV are rare complications. Cavernous transformation of the Portal vein is a condition that is seen in the setting of long standing portal vein thrombosis. There are diverse causes to be considered when chronic portal vein thrombosis leading to cavernous transformation is encountered clinically and radiologically. In this case report the predisposing condition was found to be essential thrombocythemia. The article emphasizes the importance of considering cavernous transformation of the portal vein (CTPV) as a complication in patients with essential thrombocythemia.
Keywords: Essential thrombocythemia, Portal vein thrombosis, myeloproliferative neoplasms, Cavernous transformation of the portal vein, case report.
Cite This Article:
Please cite this article in press Muhammad Hamza Mushtaq et al, Portal Vein Cavernous Transformation In A Patient With Essential Thrombocythemia: A Rare And Interesting Presentation., Indo Am. J. P. Sci, 2023; 10 (08).
Number of Downloads : 10
References:
1. Birgegård G. Long-term management of thrombocytosis in essential thrombocythaemia. Ann Hematol. 2009;88(1):1-10. doi:10.1007/s00277-008-0531-7
2. Tefferi A, Vannucchi AM, Barbui T. Essential thrombocythemia treatment algorithm 2018. Blood Cancer J. 2018;8(1):2. doi:10.1038/s41408-017-0041-8
3. Robinson AJ, Godfrey AL. Low-Risk Essential Thrombocythemia: A Comprehensive Review. HemaSphere. 2021;5(2):e521. doi:10.1097/HS9.0000000000000521
4. Ramos. Cavernous Transformation of Portal Vein Secondary to Portal Vein Thrombosis: A Case Report. J Clin Med Res. Published online 2012. doi:10.4021/jocmr775w
5. Sezgin O, Oğuz D, Altintaş E, Saritaş Ü, Şahin B. Endoscopic Management of Biliary Obstruction Caused by Cavernous Transformation of the Portal Vein. Gastrointest Endosc. 2003;58(4):602-608. doi:10.1067/S0016-5107(03)01975-8
6. Wei B, Huang Z, Tang C. Optimal Treatment for Patients With Cavernous Transformation of the Portal Vein. Front Med. 2022;9:853138. doi:10.3389/fmed.2022.853138
7. Nasim M, Majid B, Tahir F, Majid Z, Irfan I. Cavernous Transformation of Portal Vein in the Setting of Protein C and Anti-thrombin III Deficiency. Cureus. Published online September 27, 2019. doi:10.7759/cureus.5779
8. Levine RL, Wadleigh M, Cools J, et al. Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis. Cancer Cell. 2005;7(4):387-397. doi:10.1016/j.ccr.2005.03.023
9. Gioia S, Nardelli S, Ridola L, Riggio O. Causes and Management of Non-cirrhotic Portal Hypertension. Curr Gastroenterol Rep. 2020;22(12):56. doi:10.1007/s11894-020-00792-0
10. Murakami J, Shimizu Y. Hepatic Manifestations in Hematological Disorders. Int J Hepatol. 2013;2013:1-13. doi:10.1155/2013/484903
11. Cai XY, Zhou W, Hong DF, Cai XJ. A latent form of essential thrombocythemia presenting as portal cavernoma. World J Gastroenterol. 2009;15(42):5368-5370. doi:10.3748/wjg.15.5368
12. Wei B, Zhang L, Tong H, Wang Z, Wu H. Cavernous Transformation of the Portal Vein in a 26-Month Old Boy Treated by Transjugular Intrahepatic Portosystemic Shunt: A Case Report. Front Pediatr. 2019;7:379. doi:10.3389/fped.2019.00379