A case of porto-sinusoidal vascular disease
Introduction: Porto-sinusoidal vascular syndrome is characterised by specific histological changes that do not include cirrhosis, with or without portal hypertension. Patients are usually asymptomatic until development of portal hypertension complications. Case description: A 69-year-old female with...
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| Format: | Article |
| Language: | English |
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SMC MEDIA SRL
2023-10-01
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| Series: | European Journal of Case Reports in Internal Medicine |
| Subjects: | |
| Online Access: | https://www.ejcrim.com/index.php/EJCRIM/article/view/4138 |
| _version_ | 1797404171560812544 |
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| author | Luciana Silva Raquel Moura Luís Rocha Tiago Costa Filipe Breda Joana Cochicho |
| author_facet | Luciana Silva Raquel Moura Luís Rocha Tiago Costa Filipe Breda Joana Cochicho |
| author_sort | Luciana Silva |
| collection | DOAJ |
| description | Introduction: Porto-sinusoidal vascular syndrome is characterised by specific histological changes that do not include cirrhosis, with or without portal hypertension. Patients are usually asymptomatic until development of portal hypertension complications.
Case description: A 69-year-old female with history of JAK2 positive essential thrombocythemia (ET) was referred to internal medicine consultation due to elevated liver enzymes. The patient had no previous history of liver disease. Seven months earlier, she had an ischaemic stroke and started treatment with atorvastatin. After discontinuing medication, liver enzymes returned to normal and atorvastatin-related drug-induced liver disease (DILI) was presumed.
During a follow-up visit, iron deficiency anaemia was detected and an endoscopic study was performed. It revealed a gastric varix actively bleeding, which was successfully treated with cyanoacrylate.
Two months later, the patient was admitted due to a new episode of variceal bleeding, and a portal hypertension complementary study was made.
Discussion: Although the pathogenesis of porto-sinusoidal vascular disease (PSVD) remains poorly understood, vascular changes within the liver have been associated with several predisposing conditions, such as hypercoagulable states. Patients with ET, especially those with JAK2 mutation, are known to be at increased risk of non-cirrhotic vein thrombosis. Concerning PSVD, the association is not clear but it is believed that both PSVD and myeloproliferative neoplasms share a common denominator: a state characterised by hypercoagulability, inflammation, endothelial dysfunction and, in some cases, portal hypertension.
Conclusion: Portal hypertension without cirrhosis is a rare condition, presenting diagnostic challenges and significant impact on the patient’s prognosis. |
| first_indexed | 2024-03-09T02:51:04Z |
| format | Article |
| id | doaj.art-4f845adcb71b4f989453830bb04a61b5 |
| institution | Directory Open Access Journal |
| issn | 2284-2594 |
| language | English |
| last_indexed | 2024-03-09T02:51:04Z |
| publishDate | 2023-10-01 |
| publisher | SMC MEDIA SRL |
| record_format | Article |
| series | European Journal of Case Reports in Internal Medicine |
| spelling | doaj.art-4f845adcb71b4f989453830bb04a61b52023-12-05T10:10:16ZengSMC MEDIA SRLEuropean Journal of Case Reports in Internal Medicine2284-25942023-10-0110.12890/2023_0041383673A case of porto-sinusoidal vascular diseaseLuciana Silva0Raquel Moura1Luís Rocha2Tiago Costa3Filipe Breda4Joana Cochicho5Internal Medicine Department, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, PortugalInternal Medicine Department, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, PortugalInternal Medicine Department, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, PortugalInternal Medicine Department, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, PortugalInternal Medicine Department, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, PortugalInternal Medicine Department, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, PortugalIntroduction: Porto-sinusoidal vascular syndrome is characterised by specific histological changes that do not include cirrhosis, with or without portal hypertension. Patients are usually asymptomatic until development of portal hypertension complications. Case description: A 69-year-old female with history of JAK2 positive essential thrombocythemia (ET) was referred to internal medicine consultation due to elevated liver enzymes. The patient had no previous history of liver disease. Seven months earlier, she had an ischaemic stroke and started treatment with atorvastatin. After discontinuing medication, liver enzymes returned to normal and atorvastatin-related drug-induced liver disease (DILI) was presumed. During a follow-up visit, iron deficiency anaemia was detected and an endoscopic study was performed. It revealed a gastric varix actively bleeding, which was successfully treated with cyanoacrylate. Two months later, the patient was admitted due to a new episode of variceal bleeding, and a portal hypertension complementary study was made. Discussion: Although the pathogenesis of porto-sinusoidal vascular disease (PSVD) remains poorly understood, vascular changes within the liver have been associated with several predisposing conditions, such as hypercoagulable states. Patients with ET, especially those with JAK2 mutation, are known to be at increased risk of non-cirrhotic vein thrombosis. Concerning PSVD, the association is not clear but it is believed that both PSVD and myeloproliferative neoplasms share a common denominator: a state characterised by hypercoagulability, inflammation, endothelial dysfunction and, in some cases, portal hypertension. Conclusion: Portal hypertension without cirrhosis is a rare condition, presenting diagnostic challenges and significant impact on the patient’s prognosis.https://www.ejcrim.com/index.php/EJCRIM/article/view/4138porto-sinusoidal vascular diseaseportal hypertensionincomplete septal fibrosis |
| spellingShingle | Luciana Silva Raquel Moura Luís Rocha Tiago Costa Filipe Breda Joana Cochicho A case of porto-sinusoidal vascular disease European Journal of Case Reports in Internal Medicine porto-sinusoidal vascular disease portal hypertension incomplete septal fibrosis |
| title | A case of porto-sinusoidal vascular disease |
| title_full | A case of porto-sinusoidal vascular disease |
| title_fullStr | A case of porto-sinusoidal vascular disease |
| title_full_unstemmed | A case of porto-sinusoidal vascular disease |
| title_short | A case of porto-sinusoidal vascular disease |
| title_sort | case of porto sinusoidal vascular disease |
| topic | porto-sinusoidal vascular disease portal hypertension incomplete septal fibrosis |
| url | https://www.ejcrim.com/index.php/EJCRIM/article/view/4138 |
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