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

Jing WANG; Xiaofeng LIU; Jun TIE

doi:10.3969/j.issn.1001-5256.2021.11.025

Volume 37 Issue 11

Nov. 2021

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

DOI: 10.3969/j.issn.1001-5256.2021.11.025

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)

Received Date: 2021-07-26
Accepted Date: 2021-08-13
Published Date: 2021-11-20

Abstract

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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References

[1]	ABU-HILAL M, TAWAKER J. Portal hypertension secondary to myelofibrosis with myeloid metaplasia: A study of 13 cases[J]. World J Gastroenterol, 2009, 15(25): 3128-3133. DOI: 10.3748/wjg.15.3128.
[2]	POTTHOFF A, ATTIA D, PISCHKE S, et al. Long-term outcome of liver transplant patients with Budd-Chiari syndrome secondary to myeloproliferative neoplasms[J]. Liver Int, 2015, 35(8): 2042-2049. DOI: 10.1111/liv.12816.
[3]	SOKOLOVA M, TSVETAEVA N, SUKHANOVA G, et al. Ph-negative chronic myeloproliferative neoplasm (primary myelofibrosis) - as one of the reasons of the budd-Chiari syndrome[J]. Mediterr J Hematol Infect Dis, 2012, 4(1): e2012047. DOI: 10.4084/MJHID.2012.047.
[4]	CERVANTES F, BAROSI G, HERNÁNDEZ-BOLUDA JC, et al. Myelofibrosis with myeloid metaplasia in adult individuals 30 years old or younger: Presenting features, evolution and survival[J]. Eur J Haematol, 2001, 66(5): 324-327. DOI: 10.1034/j.1600-0609.2001.066005324.x.
[5]	QI X, JIA J, BAI M, et al. Portal hypertension complicating myelofibrosis in a patient without portal or hepatic vein thrombosis[J]. Ann Gastroenterol, 2014, 27(2): 188.
[6]	VAHABZADEH B, CRIPPIN JS. Image of the month. Portal hypertension as a result of extramedullary hematopoiesis caused by idiopathic myelofibrosis[J]. Clin Gastroenterol Hepatol, 2008, 6(4): e22. DOI: 10.1016/j.cgh.2008.01.008.
[7]	REILLY CR, BABUSHOK DV, MARTIN K, et al. Multicenter analysis of the use of transjugular intrahepatic portosystemic shunt for management of MPN-associated portal hypertension[J]. Am J Hematol, 2017, 92(9): 909-914. DOI: 10.1002/ajh.24798.
[8]	DOKI N, IRISAWA H, TAKADA S, et al. Transjugular intrahepatic portosystemic shunt for the treatment of portal hypertension due to idiopathic myelofibrosis[J]. Intern Med, 2007, 46(4): 187-190. DOI: 10.2169/internalmedicine.46.1768.
[9]	ALVAREZ-LARRÁN A, ABRALDES JG, CERVANTES F, et al. Portal hypertension secondary to myelofibrosis: A study of three cases[J]. Am J Gastroenterol, 2005, 100(10): 2355-2358. DOI: 10.1111/j.1572-0241.2005.50374.x.
[10]	WIEST R, STRAUCH U, WAGNER H, et al. A patient with myelofibrosis complicated by refractory ascites and portal hypertension: To tips or not to tips? A case report with discussion of the mechanism of ascites formation[J]. Scand J Gastroenterol, 2004, 39(4): 389-394. DOI: 10.1080/00365520310007521.
[11]	BĚLOHLÁVEK J, SCHWARZ J, JIRÁSEK A, et al. Idiopathic myelofibrosis complicated by portal hypertension treated with a transjugular intrahepatic portosystemic shunt (TIPS)[J]. Wien Klin Wochenschr, 2001, 113(5-6): 208-211.
[12]	HOW J, ZHOU A, OH ST. Splanchnic vein thrombosis in myeloproliferative neoplasms: Pathophysiology and molecular mechanisms of disease[J]. Ther Adv Hematol, 2017, 8(3): 107-118. DOI: 10.1177/2040620716680333.
[13]	TAN HK, LEOW WQ, CHANG PE. Ruxolitinib for the treatment of portal hypertension in a patient with primary myelofibrosis[J]. Gastroenterology, 2019, 157(5): e26-e27. DOI: 10.1053/j.gastro.2016.08.059.
[14]	PANDA A, CHANDRASHEKHARA SH, NAMBIRAJAN A, et al. Idiopathic myelofibrosis with disseminated hepatosplenic, mesenteric, renal and pulmonary extramedullary haematopoeisis, portal hypertension and tuberculosis: Initial presentation and 2 years follow-up[J]. BMJ Case Rep, 2016, 2016. DOI: 10.1136/bcr-2016-217854.
[15]	DURCHSCHEIN F, KRONES E, EHERER AJ, et al. Sclerotherapy-associated esophageal hematoma in a patient with myelofibrosis and portal hypertension[J]. Endoscopy, 2015, 47(Suppl 1)UCTN: e20-e21. DOI: 10.1055/s-0034-1390725.
[16]	TOKAI K, MIYATANI H, YOSHIDA Y, et al. Multiple esophageal variceal ruptures with massive ascites due to myelofibrosis-induced portal hypertension[J]. World J Gastroenterol, 2012, 18(28): 3770-3774. DOI: 10.3748/wjg.v18.i28.3770.
[17]	de FRANCHIS R. Expanding consensus in portal hypertension: Report of the Baveno VI Consensus Workshop: Stratifying risk and individualizing care for portal hypertension[J]. J Hepatol, 2015, 63(3): 743-752. DOI: 10.1016/j.jhep.2015.05.022.
[18]	BARBUI T, THIELE J, GISSLINGER H, et al. The 2016 WHO classification and diagnostic criteria for myeloproliferative neoplasms: Document summary and in-depth discussion[J]. Blood Cancer J, 2018, 8(2);15. DOI: 10.1038/s41408-018-0054-y.
[19]	RUMI E, CAZZOLA M. Diagnosis, risk stratification, and response evaluation in classical myeloproliferative neoplasms[J]. Blood, 2017, 129(6): 680-692. DOI: 10.1182/blood-2016-10-695957.
[20]	TEFFERI A. Primary myelofibrosis: 2019 update on diagnosis, risk-stratification and management[J]. Am J Hematol, 2018, 93(12): 1551-1560. DOI: 10.1002/ajh.25230.
[21]	SILVERSTEIN MN, WOLLAEGER EE, BAGGENSTOSS AH. Gastrointestinal and abdominal manifestations of agnogenic myeloid metaplasia[J]. Arch Intern Med, 1973, 131(4): 532-537. DOI: 10.1001/archinte.1973.00320100060007
[22]	LIGUMSKI M, POLLIACK A, BENBASSAT J. Nature and incidence of liver involvement in agnogenic myeloid metaplasia[J]. Scand J Haematol, 1978, 21(2): 81-93. DOI: 10.1111/j.1600-0609.1978.tb02497.x.
[23]	VANNUCCHI AM. Insights into the pathogenesis and management of thrombosis in polycythemia vera and essential thrombocythemia[J]. Intern Emerg Med, 2010, 5(3): 177-184. DOI: 10.1007/s11739-009-0319-3.
[24]	FUJIYAMA S, SAITOH S, KAWAMURA Y, et al. Portal vein thrombosis in liver cirrhosis: Incidence, management, and outcome[J]. BMC Gastroenterol, 2017, 17(1): 112. DOI: 10.1186/s12876-017-0668-8.
[25]	QI X, HAN G, FAN D. Management of portal vein thrombosis in liver cirrhosis[J]. Nat Rev Gastroenterol Hepatol, 2014, 11(7): 435-446. DOI: 10.1038/nrgastro.2014.36.
[26]	DELEVE LD, VALLA DC, GARCIA-TSAO G. Vascular disorders of the liver[J]. Hepatology, 2009, 49(5): 1729-1764. DOI: 10.1002/hep.22772.
[27]	SONG ZQ, ZHOU LY. A clinical analysis of six cases of portal hypertension secondary to primary myelofibrosis and review of literatures[J]. Chin J Intern Med, 2010, 49(10): 845-847. DOI: 10.3760/cma.j.issn.0578-1426.2010.10.009. 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.
[28]	WANG X, HE TT, ZHANG N, et al. Clinical characteristics of 12 patients with primary myelofibrosis and liver cirrhosis[J]. J Clin Hepatol, 2020, 36(11): 2524-2526. DOI: 10.3969/j.issn.1001-5256.2020.11.025. 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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Clinical features and prognosis of primary myelofibrosis with hepatic vascular disease and/or portal hypertension

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

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