Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2022 Sep 22:13:20406223221125691.
doi: 10.1177/20406223221125691. eCollection 2022.

Characteristics of myeloproliferative neoplasm-associated portal hypertension and endoscopic management of variceal bleeding

Xiaoquan Huang  1 , Ming Zhang  2 , Yingjie Ai  1 , Siyu Jiang  1 , Mei Xiao  3 , Lifen Wang  4 , Yourong Jian  5 , Yuzheng Zhuge  2 , Chunqing Zhang  4 , Shiyao Chen  6   7   5
Affiliations

Characteristics of myeloproliferative neoplasm-associated portal hypertension and endoscopic management of variceal bleeding

Xiaoquan Huang et al. Ther Adv Chronic Dis. .

Abstract

Background: Myeloproliferative neoplasms (MPNs) are a rare yet important clinical cause of portal hypertension, which may cause recurrent gastroesophageal variceal bleeding (GVB). MPN-associated variceal bleeding lacks specific guidelines and clinical consensus and desiderates cohort studies. We performed a multicenter retrospective study to investigate the efficacy of endoscopic management of bleeding in MPNs.

Methods: We included consecutive MPN patients with gastroesophageal varices in eight tertiary university hospitals between January 2007 and March 2020. The clinical characteristics of participants were summarized. MPN patients with a history of GVB were followed up for the rebleeding and death, compared with controls suffering from schistosomiasis-associated portal hypertension who received endoscopic treatment for variceal bleeding at the same period.

Results: A total of 62 MPN patients with gastroesophageal varices were identified, and 37 had a history of GVB. Of these, 24 patients received endoscopic variceal ligation and endoscopic injection of cyanoacrylate for the prophylaxis of variceal rebleeding. Endoscopic treatment significantly reduced the rebleeding rate in MPN patients with a history of GVB (28.2% versus 68.3%, p = 0.0269). Multivariable Cox regression indicated that endoscopic treatment (HR = 0.10, 95% CI: 0.02-0.54, p = 0.008) was the independent protective factor for decreasing the 3-year rebleeding rate, while the use of non-selective beta-blockers (NSBB) (HR = 13.41, 95% CI: 2.15-83.42, p = 0.005) was the risk factor for increasing the 3-year rebleeding rate. As for the efficacy of endoscopic management, 3-year rebleeding rate was significantly lower in MPN patients in contrast to 46 controls with schistosomiasis-associated variceal bleeding (32.9% versus 59.0%, p = 0.0346).

Conclusion: Endoscopic treatment might be a feasible and potent approach in the management of gastroesophageal variceal rebleeding in MPNs, while NSBB might be ineffective.

Keywords: Janus kinase 2; chronic hematologic malignancy; endoscopy; gastroesophageal varices; non-cirrhotic portal hypertension; rebleeding prophylaxis.

PubMed Disclaimer

Conflict of interest statement

Competing interests: The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. The article has been read and approved by all the authors.

Figures

Figure 1.
Figure 1.
Flowchart of the study.
Figure 2.
Figure 2.
(a) and (b) The portal venous phase of CT shows portal vein thrombosis and portal cavernoma. (c) and (d) Endoscopic image shows diffuse or absence of esophageal varices. (e–g) Diffuse gastric varices and thrombus on the varices and (h) endoscopic injection of cyanoacrylate.
Figure 3.
Figure 3.
Kaplan–Meier curves showing (a) the 3-year overall survival rate from the first diagnosis of gastroesophageal varices in patients with ET, PV, and PMF. (b) The 3-year overall survival rate in MPN patients with or without a history of variceal bleeding.
Figure 4.
Figure 4.
Kaplan–Meier curves showing the cumulative incidence of 3-year rebleeding rate after first bleeding episode in MPNs who underwent endoscopic treatment and those who did not undergo endoscopic treatment (p = 0.0269).
Figure 5.
Figure 5.
Kaplan–Meier curves showing the cumulative incidence of mortality rate and 3-year rebleeding rate in MPN and schistosomiasis-associated portal hypertension patients who underwent endoscopic treatment for the prevention of variceal rebleeding: (a) 3-year mortality and (b) 3-year rebleeding rate.
See this image and copyright information in PMC

Similar articles

See all similar articles

References

    1. Khanna R, Sarin SK. Non–cirrhotic portal hypertension – diagnosis and management. J Hepatol 2014; 60: 421–441. - PubMed
    1. Arber DA, Orazi A, Hasserjian R, et al.. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood 2016; 127: 2391–2405. - PubMed
    1. Zhao G, Wu ZY, Zhang B, et al.. Diagnosis and treatment of portal hypertension secondary to myeloproliferative disorders: a report of three cases. J Dig Dis 2011; 12: 312–316. - PubMed
    1. Kander EM, Raza S, Zhou Z, et al.. Bleeding complications in BCR-ABL negative myeloproliferative neoplasms: prevalence, type, and risk factors in a single-center cohort. Int J Hematol 2015; 102: 587–593. - PubMed
    1. Foucar CE, Stein BL. JAK2 V617F mutation testing in patients presenting with hepatic and portal vein thrombosis. JAMA 2017; 317: 2228–2229. - PubMed