Acute Simultaneous Thrombosis of the Hepatic Veins and the Portal System in a 30-Year-Old Female with JAK2-Positive Essential Thrombocythemia

Aim: to present a clinical case of simultaneous thrombosis of the hepatic veins and total thrombosis of the vessels of the portal system in a myeloproliferative neoplasm.

Key points. The patient was admitted to the gastroenterology department with complaints of abdominal distension, abdominal pain syndrome, and hyperthermia. An examination revealed widespread thrombosis of the hepatic veins and the portal system. Conservative therapy with diuretics, low-molecular-weight heparins, and albumin preparations proved ineffective. The patient was referred to a federal clinic for verification of the diagnosis of myeloproliferative neoplasm. As a result of the examination, a diagnosis of essential thrombocythemia was established. Against the background of comprehensive conservative therapy, a regression of refractory ascites was achieved. The patient is under dynamic observation of a hematologist, a hepatologist, hepatobiliary surgeon, and a transplantologist. 

1. Melikyan A.L., Kovrigin A.M., Subortseva I.N., Shuvaev V.A., Morozova E.V., Lomaia E.G., et al. National clinical guidelines for the diagnosis and treatment of Ph-negative myeloproliferative diseases (polycythemia vera, essential thrombocythemia, primary myelofibrosis) (2020 edition). Clinical Oncohematology. 2021;14(2):262–98. (In Russ.)]. DOI: 10.21320/2500-2139-2021-14-2-262-298

2. Tefferi A., Barbui T. Polycythemia vera and essential thrombocythemia: 2019 update on diagnosis, risk-stratification and management. Am J Hematol. 2019;94(1):133–43. DOI: 10.1002/ajh.25303

3. Meier B., Burton J.H. Myeloproliferative disorders. Hematol Oncol Clin North Am. 2017;31(6):1029–44. DOI: 10.1016/j.hoc.2017.08.007

4. de Franchis R., Bosch J., Garcia-Tsao G., Reiberger T., Ripoll C.; Baveno VII Faculty. Baveno VII — Renewing consensus in portal hypertension. J Hepatol. 2022;76(4):959–74. DOI: 10.1016/j.jhep.2021.12.022

5. Chait Y., Condat B., Cazals-Hatem D., Rufat P., Atmani S., Chaoui D., et al. Relevance of the criteria commonly used to diagnose myeloproliferative disorder in patients with splanchnic vein thrombosis. Br J Haematol. 2005;129(4):553–60. DOI: 10.1111/j.1365-2141.2005.05490.x

6. Rumi E., Cazzola E. How I treat essential thrombocythemia. Blood. 2016;128(20):2403–14. DOI: 10.1182/blood-2016-05-643346

7. Nonami T., Yokoyama I., Iwatsuki S., Starzl T.E. The incidence of portal vein thrombosis at liver transplantation. Hepatology. 1992;16(5):1195–8.

8. Bhattacharyya M., Makharia G., Kannan M., Ahmed R.P., Gupta P.K., Saxena R. Inherited prothrombotic defects in Budd-Chiari syndrome and portal vein thrombosis: A study from North India. Am J Clin Pathol. 2004;121(6):844–7. DOI: 10.1309/F2U1-XBV4-RXYUAYG0

9. Mahmoud A.E., Helmy A.S., Billingham L., Elias E. Poor prognosis and limited therapeutic options in patients with Budd-Chiari syndrome and portal venous system thrombosis. Eur J Gastroenterol Hepatol. 1997;9(5):485–9. DOI: 10.1097/00042737-199705000-00014

10. Tefferi A., Vannucchi A.M., Barbui T. Essential thrombocythemia: 2024 update on diagnosis, risk stratification, and management. Am J Hematol. 2024;99(4):697–718. DOI: 10.1002/ajh.27216

11. Tantawy A.A.-G., Adly A.A.-M., Elhenawy Y.I. Budd-Chiari syndrome complicating essential thrombocythemia in an adolescent. Blood Coagul Fibrinolysis. 2015;26(6):691–4. DOI: 10.1097/MBC.0000000000000325

12. Melikyan A.L., Sukhanova G.A., Vakhrusheva M.V., Subortseva I.N., Orel E.B. Experience in treating portal thrombosis in patients with chronic myeloproliferative diseases. Terapevticheskii Arkhiv. 2016;88(1):89–95. (In Russ.)]. DOI: 10.17116/terarkh201688189-95