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Portal hypertension secondary to myelofibrosis: a study of three cases

Alberto Alvarez-Larrán1, Juan G Abraldes, Francisco Cervantes

  • 1Hematology Department, Azienda Ospedaliero Universitaria di Careggi, Firenze, Italy.

The American Journal of Gastroenterology
|September 27, 2005
PubMed
Abstract

Insights

Idiopathic myelofibrosis (IM) can cause portal hypertension (PHT) due to presinusoidal factors, not just sinusoidal ones. Treatment for PHT complications in IM may be similar to that for cirrhosis.

Area of Science:

  • Hepatology
  • Hematology
  • Vascular Medicine

Background:

  • Portal hypertension (PHT) is uncommon in idiopathic myelofibrosis (IM) without hepatic or splenoportal vein thrombosis.
  • Investigating the underlying mechanisms of PHT in IM is crucial for effective management.

Observation:

  • Three IM patients with PHT underwent Doppler ultrasound, hemodynamic studies, and liver biopsy.
  • Two patients presented with sinusoidal PHT, normal portal flow, and significant liver infiltration.
  • One patient exhibited high portal flow and less hepatic involvement, succumbing to IM before PHT treatment.

Findings:

  • PHT in IM can stem from a significant presinusoidal component, potentially underestimated by hepatic vein catheterization.
  • Transjugular intrahepatic portosystemic shunt (TIPS) effectively managed PHT complications like variceal bleeding and ascites in two patients.
  • Massive myeloid metaplasia in the liver correlated with sinusoidal PHT.

Implications:

  • Presinusoidal PHT should be considered in IM patients, even with normal hepatic venous pressure gradients.
  • Therapeutic strategies for PHT complications in IM may mirror those used for cirrhosis.
  • Early recognition and management of PHT are vital for improving outcomes in idiopathic myelofibrosis.

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