Endoscopic treatment was shown to be an effective approach for managing gastroesophageal variceal rebleeding in patients with myeloproliferative neoplasms (MPNs). Nonselective beta-blockers (NSBBs) were not found to be as effective. These findings were published in Therapeutic Advances in Chronic Disease.

MPNs are a heterogeneous group of chronic hematologic malignancies, including polycythemia vera, essential thrombocythemia, and primary myelofibrosis. MPN is an uncommon cause of noncirrhotic portal hypertension (PHT), an early complication of which is gastroesophageal variceal bleeding (GVB) in patients with MPN. Quality of life and prognosis for these depend on symptom burden, particularly thrombotic and bleeding events.

Concerned about the lack of a clinical consensus and specific guidelines for treating MPN-associated variceal bleeding, a team of researchers explored the efficacy of endoscopic treatment recommended for variceal bleeding — endoscopic variceal ligation (EVL) and endoscopic injection of cyanoacrylate (EIC) — as prophylaxis for variceal rebleeding in patients with MPN.

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In this multicenter retrospective study, the researchers included MPN patients with gastroesophageal varices over a 13-year period at 8 tertiary university hospitals in China. Of the 62 patients with MPN who were identified, 37 had a history of GVB. Of those, 24 underwent prophylactic treatment with EVL and EIC.  Nine of the remaining 13 did not undergo any treatment, 1 underwent partial splenic embolization, 1 underwent transjugular intrahepatic protosystemic shunt (TIPS), and 2 underwent splenectomy.

Schistosomiasis-associated PHT has similar pathologic manifestation and clinical presentation, and endoscopic management for variceal bleeding is the accepted treatment. Therefore, the researchers recruited 46 patients with schistosomiasis-associated PHT as a control group.

Primary outcome measures were rebleeding and overall survival rates at 3 years after first GVB episode. All patients were followed-up by phone every 6 months until death or study termination (June 30, 2020).

Upon analysis, the researchers noted that outcomes were better for those patients without a variceal bleeding history, whereas prognosis was poor for patients with a variceal bleeding history.

However, endoscopic treatment seemed to benefit those patients with a history of bleeding. Specifically, endoscopy had a “satisfying efficacy in preventing recurrent bleeding and improving the overall survival rate” in the MPN patients with noncirrhotic PHT. The patients who underwent endoscopic treatment had a significantly lower rate of variceal rebleeding at the 3-year mark than those who did not. Efficacy was even better in patients with schistosomiasis-associated bleeding.

Combination treatment with nonselective beta-blocker (NSBB) and endoscopic treatment is recommended for patients with cirrhotic PHT experiencing recurrent variceal bleeding. However, use of NSBB seemed to have the opposite effect in noncirrhotic PHT with MPN and were found to be a risk factor for 3-year rebleeding.

These study findings show that patients with MPN might have PHT and experience variceal rebleeding. “Endoscopic treatment might be applied as the treatment of choice to prevent rebleeding in MPN patients, while NSBB might be ineffective,” the researchers concluded.

The study was limited by its relatively small sample size and retrospective nature. The researchers also noted that because the disease is rare, some patients with underlying hematologic disease may be misdiagnosed or underdiagnosed. They suggested future prospective studies could be useful in confirming the risk factor in the endoscopic management of rebleeding in MPNs.


Huang X, Zhang M, Ai Y, et al. Characteristics of myeloproliferative neoplasm-associated portal hypertension and endoscopic management of variceal bleeding. Ther Adv Chronic Dis. Published online September 22, 2202. doi:10.1177/20406223221125691