Clinical features of myeloproliferative neoplasms with portal hypertension as the main manifestation
10.3969/j.issn.1001-5256.2023.02.017
- VernacularTitle:以门静脉高压症为主要表现的骨髓增殖性肿瘤临床特征分析
- Author:
Lijuan FENG
1
;
Yan WANG
1
;
Min WANG
1
;
Guanhua ZHANG
1
;
Fuliang HE
1
;
Xinyan ZHAO
1
;
Yu WANG
1
Author Information
1. Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China
- Publication Type:Original Article_Other Liver Disease
- Keywords:
Hypertension, Portal;
Myeloproliferative Disorders;
Venous Thromboembolism
- From:
Journal of Clinical Hepatology
2023;39(2):365-369
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate the clinical features, liver histological features, and diagnostic and treatment methods for patients with myeloproliferative neoplasms (MPN) with portal hypertension as the main manifestation. Methods A retrospective analysis was performed for related data of the patients who attended the hospital due to portal hypertension and were finally diagnosed with MPN in Liver Research Center, Beijing Friendship Hospital, from January 2019 to February 2022, including clinical manifestation, liver pathological features, treatment, and follow-up results. Results Nine patients were included in this study, and all the patients had splenomegaly and esophageal and gastric varices, while portal vein thrombosis was observed in eight patients. All patients had normal or slightly abnormal liver function and routine blood test results. Six patients underwent liver biopsy, without the formation of fibrous septum and pseudolobule, and hepatic extramedullary hematopoiesis was observed in two patients. All nine patients underwent bone marrow biopsy and genetic testing, among whom six had essential thrombocythemia and three had primary myelofibrosis, and genetic testing revealed JAK - 2V617F gene mutation in seven patients and CALR gene mutation in two patients. Conclusion MPN is one of the rare causes of portal hypertension and has the clinical manifestations of esophageal and gastric varices, splenomegaly, and even megalosplenia, without the manifestations of hypersplenism such as leukopenia and thrombocytopenia. Detection of the JAK - 2V617F and CALR genes can improve the diagnostic rate of MPN.
