Analysis of the clinical characteristics of patients with myeloproliferative neoplasms complicated with portal hypertension and the therapeutic effect of ruxolitinib intervention
10.3760/cma.j.cn431274-20250321-00401
- VernacularTitle:骨髓增殖性肿瘤合并门静脉高压患者的临床特征及芦可替尼干预疗效分析
- Author:
Xuejiao ZHANG
1
;
Jinzhi XU
;
Peng LIU
Author Information
1. 复旦大学附属中山医院血液科，上海 200032
- Publication Type:Journal Article
- Keywords:
Myeloproliferative neoplasms;
Portal hypertension;
Ruxolitinib
- From:
Journal of Chinese Physician
2025;27(4):502-506
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To analyze the clinical characteristics of patients with myeloproliferative neoplasms (MPN) complicated with portal hypertension and evaluate the efficacy of ruxolitinib treatment.Methods:A total of 42 patients with MPN complicated with portal hypertension and 84 patients with MPN without portal hypertension who were admitted to the Department of Hematology of the Zhongshan Hospital, Fudan University from January 2018 to December 2023 were retrospectively included. The clinical characteristics of the two groups were compared. The MPN combined with portal hypertension group was divided into the ruxolitinib group ( n=18) and the traditional treatment group ( n=24) according to different treatment methods. The baseline characteristics and therapeutic effects of the two groups were compared. Results:Compared with patients with MPN without portal hypertension, patients with MPN combined with portal hypertension were younger (median age 49 years vs 63 years), and were more common in females [54.76%(23/42) vs 41.67%(35/84)]. The proportion of JAK2V617F mutation was higher [88.09%(37/42) vs 70.23%(59/84)], and the incidence of portal vein thrombosis was higher [57.14%(24/42) vs 1.19%(1/84)]. The incidence of gastrointestinal bleeding was high [45.24%(19/42) vs 4.76%(4/84)], and the hemoglobin and platelet counts were lower (all P<0.05). In terms of MPN classification, the proportions of PrePMF (5/18) and Post-ET-MF (4/18) were relatively high in the ruxolitinib group, while the proportions of essential thrombocythemia (ET) [41.67%(10/24)] and polycythemia vera (PV) [25.00%(6/24)] were relatively high in the traditional treatment group. The distribution of driver genes showed that the JAK2V617F mutation rate in the ruxolitinib group was higher than that in the traditional treatment group (all P<0.05). The proportion of patients with spleen volume reduction ≥35%(SVR35) in the ruxolitinib group was 4/18, and that in the traditional treatment group was 8.33%(2/24), with no statistically significant difference ( P=0.21). The portal vein diameter in the ruxolitinib group decreased compared with the baseline [(14.2±2.1)mm vs (16.5±2.8)mm, P=0.02], while no improvement was observed in the traditional treatment group. Conclusions:Ruxolitinib can effectively improve the portal vein diameter of patients with MPN complicated with portal hypertension.
