原发性骨髓纤维化合并肝血管性疾病和/或门静脉高压症的临床特征和预后

王静; 刘晓峰; 帖君

doi:10.3969/j.issn.1001-5256.2021.11.025

原发性骨髓纤维化合并肝血管性疾病和/或门静脉高压症的临床特征和预后

DOI: 10.3969/j.issn.1001-5256.2021.11.025

王静^{1, 2},
刘晓峰^1, ,,
帖君^2, ,

1.
中国人民解放军联勤保障部队第九六〇医院消化内科，济南 250000
2.
空军军医大学西京消化病医院介入中心，西安 710032

基金项目:

国家自然科学基金 (81900467);

解放军第九六〇医院院长基金资助项目 (2018ZD02)

详细信息

通信作者:
刘晓峰，liuxf0531@126.com

帖君，tiejun7776@163.com

中图分类号: R575.2
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出版历程
- 收稿日期: 2021-07-26
- 录用日期: 2021-08-13
- 出版日期: 2021-11-20

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

Jing WANG^{1, 2},
Xiaofeng LIU^{1
, ,},
Jun TIE^{2
, ,}

1.
Department of Gastroenterology, 960 Hospital of the PLA Joint Logistics Support Force, Jinan 250000, China
2.
Department of Digestive Interventional Radiology, Xijing Hospital of Digestive Diseases, Air Force Medical University, Xi'an 710032, China

Research funding:

National Natural Science Foundation of China (81900467);

the 960th Hospital Dean's Fund Project (2018ZD02)

摘要

摘要: 目的探讨原发性骨髓纤维化(PMF)相关肝血管疾病和门静脉高压症的临床特征、肝组织病理学特点和预后。方法选取2010年7月—2020年12月在解放军第九六〇医院和空军军医大学西京消化病医院确诊的68例PMF患者，其中22例以肝血管疾病/门静脉高压症为主要表现，回顾性分析了这些患者的临床和病理特征。根据患者有无血栓形成分为两组，总结其治疗和预后情况。通过Kaplan-Meier绘制生存曲线，通过log-rank检验比较两组间长期生存率。结果 68例PMF患者中，22例出现肝血管疾病和/或门静脉高压症，患病率约为32.35%。其中，13例(59.1%)表现为门静脉血栓形成，1例(4.5%)表现为Budd-Chiari综合征，8例(36.4%)表现为门静脉高压症。7例患者接受肝活检，病理均显示肝脏髓外造血，小叶和汇管区淋巴细胞、浆细胞和嗜酸性粒细胞不同程度浸润，但肝小叶结构正常。随访期间7例死亡患者，其中5例死于血栓形成或门静脉高压相关并发症。患者总体中位生存时间为57.99个月，其中血栓形成组中位生存时间为45.33个月，无血栓组中位生存时间为64.00个月，尽管没有显著统计学差异(χ²=3.035，P=0.081)，但是可以看到非血栓组较血栓组表现出更好的生存趋势。结论肝血管疾病和门静脉高压症患者应考虑原发病为PMF的可能性，而PMF患者也应进行肝血管疾病筛查，并早期干预。无血栓形成的患者可能比血栓形成的患者有更好的生存趋势。
- 原发性骨髓纤维化 /
- 高血压, 门静脉 /
- 静脉血栓形成 /
- 治疗学 /
- 预后
Abstract: Objective To investigate the clinical features, liver histopathological features, treatment, and prognosis of primary myelofibrosis (PMF)-associated hepatic vascular disease and portal hypertension. Methods A 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. Results Among 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. Conclusion The 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.
- Primary Myelofibrosis /
- Hypertension, Portal /
- Venous Thrombosis /
- Therapeutics /
- Prognosis

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图 1 3例典型患者的肝组织HE染色结果(×100)

注：a，肝脏髓外造血，如箭头所示；b，肝脏结节性再生性增生，如箭头所示；c，肝门区域纤维化。患者肝小叶结构正常，无肝硬化迹象。

下载: 全尺寸图片幻灯片

图 2 血栓组与非血栓组的总体生存率分析

下载: 全尺寸图片幻灯片

表 1 22例PMF并发肝血管疾病/门静脉高压症患者的临床特征

变量	数值
年龄(岁)	54(23~77)
性别[例(%)]
男性	9 (40.91)
女性	13(59.09)
门静脉高压症的病因(例)
Budd-Chiari综合征	1
PVT	13
单纯门静脉高压症	8
初诊症状(例)
食管胃底静脉曲张出血	3
腹水	7
食管胃底静脉曲张出血+腹水	3
腹痛	5
黄疸	1
乏力	3
脾肿大(例)	22
肝肿大(例)	3
血常规
血红蛋白(g/dL)	103 (56~156)
白细胞(×10⁹/L)	11.14 (1.37~38.15)
血小板(×10⁹/L)	201 (17~1028)
肝功能
AST (U/L)	23 (6~53)
ALT (U/L)	14 (6~69)
总胆红素(mg/dL)	18.6 (8.8~85.0)
凝血时间(s)	15 (13~18)
白蛋白(g/dL)	39.6 (28.4~46.1)
基因突变检测[例(%)]
JAK2	8/14 (57.14)
CALR	3/11 (27.27)
MTHFR(SNP)	6/7 (85.71)

下载: 导出CSV

表 2 PMF并发肝血管疾病/门静脉高压症患者治疗情况

治疗情况	例数	血栓组(n=14)	非血栓组(n=8)
抗凝和溶栓	11	11	0
NSBB	2	0	2
TIPS	5	2	3
Ruxolitinib	1	0	1
未治疗	3	1	2

下载: 导出CSV

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