Hepatoportal Sclerosis—A Clinicopathologic Review of 28 Cases
Background and Aims: The aim of this study was to review a large series of cases with hepatoportal sclerosis (HPS) as a pathologically recognizable entity in liver tissue specimens and describe the associated clinical and radiographic manifestations, along with the outcomes of this entity. Methods:...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
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Elsevier
2023-01-01
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| Series: | Gastro Hep Advances |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2772572323000031 |
| _version_ | 1797843866149191680 |
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| author | Nazia Khatoon Andrew P. Keaveny Gian P. Carames Murli Krishna Jason Lewis Raouf E. Nakhleh |
| author_facet | Nazia Khatoon Andrew P. Keaveny Gian P. Carames Murli Krishna Jason Lewis Raouf E. Nakhleh |
| author_sort | Nazia Khatoon |
| collection | DOAJ |
| description | Background and Aims: The aim of this study was to review a large series of cases with hepatoportal sclerosis (HPS) as a pathologically recognizable entity in liver tissue specimens and describe the associated clinical and radiographic manifestations, along with the outcomes of this entity. Methods: Data were collected through a retrospective chart review. Results: Twenty-eight patients were identified that had pathologically defined HPS. All patients had a significant past medical history and signs and symptoms of portal hypertension. The most consistent laboratory finding was an elevated alkaline phosphatase. Radiographically, 9 patients were mistakenly identified as having advanced fibrosis/cirrhosis. The initial histologic diagnosis was made on biopsy in 20 patients and after transplant in 8 patients. The severity of symptoms was variable and required transplantation in 11 patients, 3 were treated with transjugular intrahepatic portosystemic shunt, and the remaining patients were treated symptomatically. Conclusion: HPS is associated with past medical history that may be causal in nature. Signs and symptoms may be severe enough to require liver transplantation. A significant proportion of patients are radiographically misdiagnosed as cirrhosis. In this small series, overall outcomes for transplanted patients are acceptable. |
| first_indexed | 2024-04-09T17:12:57Z |
| format | Article |
| id | doaj.art-a81178b17284452b999378d0b6484dc4 |
| institution | Directory Open Access Journal |
| issn | 2772-5723 |
| language | English |
| last_indexed | 2024-04-09T17:12:57Z |
| publishDate | 2023-01-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Gastro Hep Advances |
| spelling | doaj.art-a81178b17284452b999378d0b6484dc42023-04-20T04:37:59ZengElsevierGastro Hep Advances2772-57232023-01-0123303306Hepatoportal Sclerosis—A Clinicopathologic Review of 28 CasesNazia Khatoon0Andrew P. Keaveny1Gian P. Carames2Murli Krishna3Jason Lewis4Raouf E. Nakhleh5Department of Laboratory Medicine and Pathology, Mayo Clinic, Jacksonville, FloridaDivision of Gastroenterology and Hepatology, Department of Transplant Medicine, Mayo Clinic, Jacksonville, FloridaDepartment of Laboratory Medicine and Pathology, Mayo Clinic, Jacksonville, FloridaDepartment of Laboratory Medicine and Pathology, Mayo Clinic, Jacksonville, FloridaDepartment of Laboratory Medicine and Pathology, Mayo Clinic, Jacksonville, FloridaDepartment of Laboratory Medicine and Pathology, Mayo Clinic, Jacksonville, Florida; Correspondence: Address correspondence to: Raouf E. Nakhleh, MD, Department of Laboratory Medicine and Pathology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, Florida 32224.Background and Aims: The aim of this study was to review a large series of cases with hepatoportal sclerosis (HPS) as a pathologically recognizable entity in liver tissue specimens and describe the associated clinical and radiographic manifestations, along with the outcomes of this entity. Methods: Data were collected through a retrospective chart review. Results: Twenty-eight patients were identified that had pathologically defined HPS. All patients had a significant past medical history and signs and symptoms of portal hypertension. The most consistent laboratory finding was an elevated alkaline phosphatase. Radiographically, 9 patients were mistakenly identified as having advanced fibrosis/cirrhosis. The initial histologic diagnosis was made on biopsy in 20 patients and after transplant in 8 patients. The severity of symptoms was variable and required transplantation in 11 patients, 3 were treated with transjugular intrahepatic portosystemic shunt, and the remaining patients were treated symptomatically. Conclusion: HPS is associated with past medical history that may be causal in nature. Signs and symptoms may be severe enough to require liver transplantation. A significant proportion of patients are radiographically misdiagnosed as cirrhosis. In this small series, overall outcomes for transplanted patients are acceptable.http://www.sciencedirect.com/science/article/pii/S2772572323000031Portal HypertensionPorto-Sinusoidal Vascular DisorderNon-Cirrhotic Portal HypertensionTransplantation |
| spellingShingle | Nazia Khatoon Andrew P. Keaveny Gian P. Carames Murli Krishna Jason Lewis Raouf E. Nakhleh Hepatoportal Sclerosis—A Clinicopathologic Review of 28 Cases Gastro Hep Advances Portal Hypertension Porto-Sinusoidal Vascular Disorder Non-Cirrhotic Portal Hypertension Transplantation |
| title | Hepatoportal Sclerosis—A Clinicopathologic Review of 28 Cases |
| title_full | Hepatoportal Sclerosis—A Clinicopathologic Review of 28 Cases |
| title_fullStr | Hepatoportal Sclerosis—A Clinicopathologic Review of 28 Cases |
| title_full_unstemmed | Hepatoportal Sclerosis—A Clinicopathologic Review of 28 Cases |
| title_short | Hepatoportal Sclerosis—A Clinicopathologic Review of 28 Cases |
| title_sort | hepatoportal sclerosis a clinicopathologic review of 28 cases |
| topic | Portal Hypertension Porto-Sinusoidal Vascular Disorder Non-Cirrhotic Portal Hypertension Transplantation |
| url | http://www.sciencedirect.com/science/article/pii/S2772572323000031 |
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