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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

王静,刘晓峰,帖君. 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[J]. 临床肝胆病杂志, 2021, 37(11): 2617-2621. DOI: 10.3969/j.issn.1001-5256.2021.11.025.
引用本文: 王静,刘晓峰,帖君. 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[J]. 临床肝胆病杂志, 2021, 37(11): 2617-2621. DOI: 10.3969/j.issn.1001-5256.2021.11.025.
WANG J, LIU XF, TIE J. Clinical features and prognosis of primary myelofibrosis with hepatic vascular disease and/or portal hypertension[J]. J Clin Hepatol, 2021, 37(11): 2617-2621. DOI: 10.3969/j.issn.1001-5256.2021.11.025.
Citation: WANG J, LIU XF, TIE J. Clinical features and prognosis of primary myelofibrosis with hepatic vascular disease and/or portal hypertension[J]. J Clin Hepatol, 2021, 37(11): 2617-2621. 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
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

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

Research funding: 

National Natural Science Foundation of China 81900467;

the 960th Hospital Dean's Fund Project 2018ZD02

  • 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。

     

  • 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。

    图  2  血栓组与非血栓组的总体生存率分析

    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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    宋志强, 周丽雅. 原发性骨髓纤维化继发门脉高压症六例分析并文献复习[J]. 中华内科杂志, 2010, 49(10): 845-847. DOI: 10.3760/cma.j.issn.0578-1426.2010.10.009.
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    王宣, 何婷婷, 张宁, 等. 12例原发性骨髓纤维化合并肝硬化患者的临床特征分析[J]. 临床肝胆病杂志, 2020, 36(11): 2524-2526. DOI: 10.3969/j.issn.1001-5256.2020.11.025.
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  • 收稿日期:  2021-07-26
  • 录用日期:  2021-08-13
  • 出版日期:  2021-11-20
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