Segmental portal hypertension and polycythemia vera

Area of Science:

• Hematology
• Gastroenterology
• Vascular Surgery

Background:

• Polycythemia vera (PV) is a myeloproliferative neoplasm characterized by increased red blood cell production.
• Gastroesophageal varices are swollen veins in the esophagus, often caused by portal hypertension.
• Splenic vein thrombosis is a blockage in the vein that drains the spleen.

Observation:

• A patient with known polycythemia vera presented with recurrent melena (dark, tarry stools).
• The patient was diagnosed with bleeding gastroesophageal varices.
• Angiography indicated splenic vein thrombosis.

Findings:

• The patient underwent splenectomy (surgical removal of the spleen).
• Following splenectomy, the gastroesophageal varices disappeared.
• There was no recurrence of bleeding after the surgery.

Implications:

• This case represents the first described instance of polycythemia vera associated with segmental portal hypertension.
• It is proposed that polycythemia vera patients may develop asymptomatic (silent) segmental portal hypertension.
• Clinicians should consider the possibility of silent segmental portal hypertension in polycythemia vera patients, particularly when managing gastrointestinal bleeding.