CLINICAL OBSERVATION OF A PATIENT WITH PORTAL BILIOPATHY AGAINST THE BACKGROUND OF POLYCYTHEMIA VERA

  • O.A. Kizimova Saint Petersburg State Pediatric Medical University. Lithuania 2, Saint Petersburg, Russian Federation, 194100
  • Yu.A. Fominykh Saint Petersburg State Pediatric Medical University. Lithuania 2, Saint Petersburg, Russian Federation, 194100
  • A.V. Shotik First Saint-Petersburg State Medical University named after I.P. Pavlov. Leo Tolstoy str., 6–8. Saint Petersburg, Russia, 197022
  • Yu.P. Uspenskiy Saint Petersburg State Pediatric Medical University. Lithuania 2, Saint Petersburg, Russian Federation, 194100
  • A.Sh. Mamedova Paediatrician outpatient clinic № 8, DPO № 58. Iskrovskiy pr., 8, Saint Petersburg, Russian Federation, 193615
Keywords: myeloproliferative disease, polycythemia vera, portal biliopathy, portal hypertension, portal vein thrombosis

Abstract

Myeloproliferative diseases are dangerous for the development of arterial and venous thrombosis, leading to disability and death of patients. Thrombotic complications have different localization. Portal vein thrombosis is often characterized by an asymptomatic clinical picture, which makes timely diagnosis difficult and leads to a protracted course and the development of complications. This article presents a rare clinical case of portal biliopathy in a patient with polycythemia vera. A feature of clinical observation is a pronounced cholestasis syndrome in a patient as a result of the development of portal biliopathy against the background of portal vein thrombosis. The appointment of anticoagulant therapy led to an improvement in the clinical and laboratory picture. Thus, we would like to emphasize the need to perform ultrasound duplex scanning of the celiac trunk as an initial diagnosis in patients with myeloproliferative disease and unverified hepatitis.

References

Брус Т.В., Васильев А.Г. Современное представление о неалкогольной жировой болезни печени. Russian Biomedical Research. 2020; 5(1): 18–25.

Брус Т.В., Евграфов В.А. Патофизиология печеночной недостаточности. Педиатр. 2022; 13(3): 55–64. DOI: 10.17816/PED13355-64.

Лазебник Л.Б., Голованова Е.В., Туркина С.В. и др. Неалкогольная жировая болезнь печени у взрослых: клиника, диагностика, лечение. Рекомендации для терапевтов, третья версия. Экспериментальная и клиническая гастроэнтерология. 2021; 1(185): 4–52. DOI: 10.31146/1682-8658-ecg-185-1-4-52.

Меликян А.Л., Ковригина А.М., Суборцева И.Н. и др. Национальные клинические рекомендации по диагностике и лечению Ph-негативных миелопролиферативных заболеваний (истинной полицитемии, эссенциальной тромбоцитемии, первичного миелофиброза) (редакция 2020 г.). Клиническая онкогематология. 2021; 14(2): 262–98.

Успенский Ю.П., Фоминых Ю.А., Наджафова К.Н. Липидный статус, микробиота и желчные кислоты: клинико-патогенетические взаимосвязи. Университетский терапевтический вестник. 2022; 4(2): 4–13.

Gebreselassie A., Bukhari M., Awan A. et al. Recurrent Biliary Obstruction Secondary to Portal Biliopathy and the Role of Cholangioscopy: A Case Report [corrected]. Cureus. 2018; 10(1): e2046. DOI: 10.7759/cureus.2046. Erratum in: Cureus. 2018; 10 (3): 11. PMID: 29541567; PMCID: PMC5844596.

Griesshammer M., Kiladjian J.J., Besses C. Thromboembolic events in polycythemia vera. Ann Hematol. 2019; 98(5): 1071–82. DOI: 10.1007/s00277-019-03625-x. PMID: 30848334; PMCID: PMC6469649.

Hulshoff J.B., Cuperus F.J.C., de Haas R.J. Mass-For­ming. Portal Biliopathy Presenting as Extreme Wall-Thickening of the Common Bile Duct. Diagnostics (Basel). 2020; 10(9): 623. DOI: 10.3390/diagnostics10090623. PMID: 32842665; PMCID: PMC7554880.

Khan A., Uzoka K., Macdonald S. et al. Portal Bilio­pathy: A Review of Imaging Features of Nine Patients. International Journal of Clinical Medicine. 2017; 8: 604–17. DOI: 10.4236/ijcm.2017.811057.

LiverTox: Clinical and Research Information on Drug-Induced Liver Injury [Internet]. Available at: https://www.ncbi.nlm.nih.gov/books/NBK548098/ (accessed 01.03.2023).

LiverTox: Clinical and Research Information on Drug-Induced Liver Injury [Internet]. Available at: https://www.ncbi.nlm.nih.gov/books/NBK548724/ (accessed 01.03.2023).

Lu X., Chang R. Polycythemia Vera. 2022 Apr 28. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023; PMID: 32491592.

Ramos R., Park Y., Shazad G. et al. Cavernous transformation of portal vein secondary to portal vein thrombosis: a case report. J Clin Med Res. 2012; 4(1): 81–4. DOI: 10.4021/jocmr775w. PMID: 22383935; PMCID: PMC3279509.

Wei B., Huang Z., Tang C. Optimal Treatment for Patients With Cavernous Transformation of the Portal Vein. Front Med (Lausanne). 2022; 9: 853138. DOI: 10.3389/fmed.2022.853138. PMID: 35402447; PMCID: PMC8987530.

REFERENCES

Brus T.V., Vasil’ev A.G. Sovremennoe predstavlenie o nealkogol’noj zhirovoj bolezni pecheni. [Modern understanding of non-alcoholic fatty liver disease]. Russian Biomedical Research. 2020; 5(1): 18–25. (in Russian).

Brus T.V., Evgrafov V.A. Patofiziologiya pechenochnoj nedostatochnosti. [Pathophysiology of liver fai­lure]. Pediatr. 2022; 13(3): 55–64. DOI: 10.17816/PED13355-64. (in Russian).

Lazebnik L.B., Golovanova E.V., Turkina S.V. i dr. Nealkogol'naya zhirovaya bolezn' pecheni u vzroslyh: klinika, diagnostika, lechenie. Rekomendacii dlya terapevtov, tret'ya versiya. [Non-alcoholic fatty liver disease in adults: clinic, diagnosis, treatment. Recommendations for therapists, the third version]. Eksperimental'naya i klinicheskaya gastroenterologiya. 2021; 1(185): 4–52. DOI: 10.31146/1682-8658-ecg-185-1-4-52. (in Russian).

Melikjan A.L., Kovrigina A.M., Suborceva I.N. et al. Nacional'nye klinicheskie rekomendacii po diagnostike i lecheniju Ph-negativnyh mieloproliferativnyh zabolevanij (istinnoj policitemii, jessencial'noj trombocitemii, pervichnogo mielofibroza) (redakcija 2020 g.). Klinicheskaja onkogematologija. 2021; 14(2): 262–98. (in Russian).

Uspenskiy Yu.P., Fominyh Yu.A., Nadzhafova K.N. Lipidnyj status, mikrobiota i zhelchnye kisloty: kliniko-patogeneticheskie vzaimosvyazi. [Lipid status, microbiota and bile acids: clinical and pathogenetic relationships]. Universitetskij terapevticheskij vestnik. 2022; 4(2): 4–13. (in Russian).

Gebreselassie A., Bukhari M., Awan A. et al. Recurrent Biliary Obstruction Secondary to Portal Biliopathy and the Role of Cholangioscopy: A Case Report [corrected]. Cureus. 2018; 10(1): e2046. DOI: 10.7759/cureus.2046. Erratum in: Cureus. 2018; 10 (3): 11. PMID: 29541567; PMCID: PMC5844596.

Griesshammer M., Kiladjian J.J., Besses C. Thromboembolic events in polycythemia vera. Ann Hematol. 2019; 98(5): 1071–82. DOI: 10.1007/s00277-019-03625-x. PMID: 30848334; PMCID: PMC6469649.

Hulshoff J.B., Cuperus F.J.C., de Haas R.J. Mass-For­ming Portal Biliopathy Presenting as Extreme Wall-Thickening of the Common Bile Duct. Diagnostics (Basel). 2020; 10(9): 623. DOI: 10.3390/diagnostics10090623. PMID: 32842665; PMCID: PMC7554880.

Khan A., Uzoka K., Macdonald S. et al. Portal Bilio­pathy: A Review of Imaging Features of Nine Patients. International Journal of Clinical Medicine. 2017; 8: 604–17. DOI: 10.4236/ijcm.2017.811057.

LiverTox: Clinical and Research Information on Drug-Induced Liver Injury [Internet]. Available at: https://www.ncbi.nlm.nih.gov/books/NBK548098/ (accessed 01.03.2023).

LiverTox: Clinical and Research Information on Drug-Induced Liver Injury [Internet]. Available at: https://www.ncbi.nlm.nih.gov/books/NBK548724/ (accessed 01.03.2023).

Lu X., Chang R. Polycythemia Vera. 2022 Apr 28. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023; PMID: 32491592.

Ramos R., Park Y., Shazad G. et al. Cavernous transformation of portal vein secondary to portal vein thrombosis: a case report. J Clin Med Res. 2012; 4(1): 81–4. DOI: 10.4021/jocmr775w. PMID: 22383935; PMCID: PMC3279509.

Wei B., Huang Z., Tang C. Optimal Treatment for Patients With Cavernous Transformation of the Portal Vein. Front Med (Lausanne). 2022; 9: 853138. DOI: 10.3389/fmed.2022.853138. PMID: 35402447;

PMCID: PMC8987530.

Published
2023-12-28
How to Cite
Kizimova, O., Fominykh, Y., Shotik, A., Uspenskiy, Y., & Mamedova, A. (2023). CLINICAL OBSERVATION OF A PATIENT WITH PORTAL BILIOPATHY AGAINST THE BACKGROUND OF POLYCYTHEMIA VERA. University Therapeutic Journal, 5(3), 144-156. https://doi.org/10.56871/UTJ.2023.85.51.015
Section
Статьи