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Myelofibrosis and Portal Hypertension: The Case for Primary Variceal Screening

Marc Samuel Sherman1, Wesley R Samore2, Daniel Stephan Pratt1

  • 1Division of Gastroenterology, Massachusetts General Hospital and Harvard Medical School, Boston, MA.

ACG Case Reports Journal
|May 23, 2020
PubMed

Insights

Myelofibrosis can cause portal hypertension and bleeding, even without clots. Patients with myelofibrosis may benefit from regular screening for esophageal varices.

Area of Science:

  • Hematology
  • Gastroenterology
  • Vascular Medicine

Background:

  • Myelofibrosis is a myeloproliferative neoplasm associated with bone marrow fibrosis.
  • Portal venous clots are a known complication, potentially leading to noncirrhotic portal hypertension.
  • Extramedullary hematopoiesis is common in myelofibrosis.

Observation:

  • A case of myelofibrosis with portal hypertension and extramedullary hematopoiesis is presented.
  • This patient lacked imaging or pathological evidence of portal venous clots (microvascular or macrovascular).

Findings:

  • Literature review indicates a significant incidence of portal hypertension and variceal bleeding in myelofibrosis, polycythemia vera, and essential thrombocytosis.
  • Portal hypertension in myelofibrosis may occur independently of identifiable portal venous thrombosis.

Implications:

  • Patients with myelofibrosis and related disorders are at risk for portal hypertension and its complications.
  • Primary screening for esophageal varices is proposed for this patient population.
  • Further research into the mechanisms of noncirrhotic portal hypertension in myelofibrosis is warranted.

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