Clinical features and prognosis of primary myelofibrosis with hepatic vascular disease and/or portal hypertension

ObjectiveTo investigate the clinical features, liver histopathological features, treatment, and prognosis of primary myelofibrosis (PMF)-associated hepatic vascular disease and portal hypertension. MethodsA retrospective analysis was performed for the clinical and pathological features of 68 patient...

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Autor principal:	WANG Jing
Formato:	article
Lenguaje:	ZH
Publicado:	Editorial Department of Journal of Clinical Hepatology 2021
Materias:	Diseases of the digestive system. Gastroenterology RC799-869
Acceso en línea:	https://doaj.org/article/1ddaf42d065e4c8090e0eab681d138a0
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spelling	oai:doaj.org-article:1ddaf42d065e4c8090e0eab681d138a02021-11-17T11:07:52ZClinical features and prognosis of primary myelofibrosis with hepatic vascular disease and/or portal hypertension1001-5256https://doaj.org/article/1ddaf42d065e4c8090e0eab681d138a02021-11-01T00:00:00Zhttp://www.lcgdbzz.org/cn/article/doi/10.3969/j.issn.1001-5256.2021.11.025https://doaj.org/toc/1001-5256ObjectiveTo investigate the clinical features, liver histopathological features, treatment, and prognosis of primary myelofibrosis (PMF)-associated hepatic vascular disease and portal hypertension. MethodsA retrospective analysis was performed for the clinical and pathological features of 68 patients who were diagnosed with PMF in 960 Hospital of the PLA Joint Logistics Support Force and Xijing Hospital of Digestive Diseases, Air Force Medical University, from July 2010 to December 2020, among whom 22 patients had hepatic vascular disease/portal hypertension as the main manifestation. The patients were divided into two groups according to the presence or absence of thrombosis, and treatment and prognosis were summarized. The Kaplan-Meier method was used to plot survival curves, and the log-rank test was used to compare long-term survival rate between the two groups. ResultsAmong the 68 patients with PMF, 22 had hepatic vascular disease and/or portal hypertension, resulting in a prevalence rate of 32.35%, and among these 22 patients, 13 (59.1%) had extrahepatic portal vein occlusion, 1 (4.5%) had Budd-Chiari syndrome, and 8 (36.4%) had portal hypertension. Biopsy was performed for 7 patients, and pathological results showed extramedullary hematopoiesis in the liver and varying degrees of infiltration of lymphocytes, plasma cells, and eosinophils at the lobular and portal areas, but the lobular structure was normal. A total of 7 patients died during follow-up, among whom 5 died of complications associated with thrombosis or portal hypertension. The overall median survival time was 57.99 months for all patients; the median survival time was 45.33 months in patients with thrombosis and 64.00 months in patients without thrombosis, and although there was no significant difference between the two groups (χ2=3.035, P=0.081), the non-thrombosis group tended to have better survival and prognosis than the thrombosis group. ConclusionThe possibility of PMF as the primary disease should be considered for patients with hepatic vascular disease and portal hypertension. Patients with PMF should be screened for hepatic vascular disease, and early intervention should be given. The patients without thrombosis tend to have better survival and prognosis than those with thrombosis.WANG JingEditorial Department of Journal of Clinical HepatologyarticleDiseases of the digestive system. GastroenterologyRC799-869ZHLinchuang Gandanbing Zazhi, Vol 37, Iss 11, Pp 2617-2621 (2021)
institution	DOAJ
collection	DOAJ
language	ZH
topic	Diseases of the digestive system. Gastroenterology RC799-869
spellingShingle	Diseases of the digestive system. Gastroenterology RC799-869 WANG Jing Clinical features and prognosis of primary myelofibrosis with hepatic vascular disease and/or portal hypertension
description	ObjectiveTo investigate the clinical features, liver histopathological features, treatment, and prognosis of primary myelofibrosis (PMF)-associated hepatic vascular disease and portal hypertension. MethodsA retrospective analysis was performed for the clinical and pathological features of 68 patients who were diagnosed with PMF in 960 Hospital of the PLA Joint Logistics Support Force and Xijing Hospital of Digestive Diseases, Air Force Medical University, from July 2010 to December 2020, among whom 22 patients had hepatic vascular disease/portal hypertension as the main manifestation. The patients were divided into two groups according to the presence or absence of thrombosis, and treatment and prognosis were summarized. The Kaplan-Meier method was used to plot survival curves, and the log-rank test was used to compare long-term survival rate between the two groups. ResultsAmong the 68 patients with PMF, 22 had hepatic vascular disease and/or portal hypertension, resulting in a prevalence rate of 32.35%, and among these 22 patients, 13 (59.1%) had extrahepatic portal vein occlusion, 1 (4.5%) had Budd-Chiari syndrome, and 8 (36.4%) had portal hypertension. Biopsy was performed for 7 patients, and pathological results showed extramedullary hematopoiesis in the liver and varying degrees of infiltration of lymphocytes, plasma cells, and eosinophils at the lobular and portal areas, but the lobular structure was normal. A total of 7 patients died during follow-up, among whom 5 died of complications associated with thrombosis or portal hypertension. The overall median survival time was 57.99 months for all patients; the median survival time was 45.33 months in patients with thrombosis and 64.00 months in patients without thrombosis, and although there was no significant difference between the two groups (χ2=3.035, P=0.081), the non-thrombosis group tended to have better survival and prognosis than the thrombosis group. ConclusionThe possibility of PMF as the primary disease should be considered for patients with hepatic vascular disease and portal hypertension. Patients with PMF should be screened for hepatic vascular disease, and early intervention should be given. The patients without thrombosis tend to have better survival and prognosis than those with thrombosis.
format	article
author	WANG Jing
author_facet	WANG Jing
author_sort	WANG Jing
title	Clinical features and prognosis of primary myelofibrosis with hepatic vascular disease and/or portal hypertension
title_short	Clinical features and prognosis of primary myelofibrosis with hepatic vascular disease and/or portal hypertension
title_full	Clinical features and prognosis of primary myelofibrosis with hepatic vascular disease and/or portal hypertension
title_fullStr	Clinical features and prognosis of primary myelofibrosis with hepatic vascular disease and/or portal hypertension
title_full_unstemmed	Clinical features and prognosis of primary myelofibrosis with hepatic vascular disease and/or portal hypertension
title_sort	clinical features and prognosis of primary myelofibrosis with hepatic vascular disease and/or portal hypertension
publisher	Editorial Department of Journal of Clinical Hepatology
publishDate	2021
url	https://doaj.org/article/1ddaf42d065e4c8090e0eab681d138a0
work_keys_str_mv	AT wangjing clinicalfeaturesandprognosisofprimarymyelofibrosiswithhepaticvasculardiseaseandorportalhypertension
_version_	1718425594657505280

Clinical features and prognosis of primary myelofibrosis with hepatic vascular disease and/or portal hypertension

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