From the Guidelines
Myelofibrosis patients with portal hypertension should be managed with a focus on treating the underlying myelofibrosis and its complications, including the use of hydroxyurea for splenomegaly and cytoreduction, as recommended by the European LeukemiaNet 1. The management of myelofibrosis and portal hypertension involves a multi-faceted approach, considering the patient's overall clinical condition and the severity of their disease.
- Primary myelofibrosis can lead to portal hypertension through several mechanisms, including bone marrow fibrosis, extramedullary hematopoiesis, and portal vein thrombosis.
- The European LeukemiaNet recommends the use of hydroxyurea as the first-line treatment for primary myelofibrosis-associated splenomegaly 1.
- Indications for splenectomy in myelofibrosis patients include symptomatic portal hypertension, drug-refractory painful splenomegaly, and frequent RBC transfusions 1.
- Patients with myelofibrosis and portal hypertension may also experience other complications, such as ascites, variceal bleeding, and thrombohemorrhagic events, which require prompt management 1.
- The prognosis for myelofibrosis patients with portal hypertension depends on the stage of their disease and the severity of their portal hypertension, with allogeneic stem cell transplantation being a potentially curative option for eligible patients 1.
- Regular monitoring of complete blood counts, liver function, and endoscopic surveillance for varices is essential in the management of myelofibrosis and portal hypertension.
- The use of beta-blockers, such as propranolol or nadolol, may be considered to reduce portal pressure, and diuretics like spironolactone and furosemide can be used to manage ascites.
- In severe cases, transjugular intrahepatic portosystemic shunt (TIPS) may be considered as a treatment option.
From the Research
Myelofibrosis and Portal Hypertension
- Myelofibrosis is a hematologic condition that can lead to the formation of large and small portal venous clots, resulting in portal hypertension 2.
- Portal injury is believed to underlie the mechanism of development of noncirrhotic portal hypertension in patients with myelofibrosis 2.
- Patients with myelofibrosis and related conditions, such as polycythemia vera and essential thrombocytosis, may present with portal hypertension and variceal bleeding 2.
Treatment and Management
- The treatment of myelofibrosis-related portal hypertension may involve the use of cytoreductive agents, such as hydroxyurea, to control splenomegaly and reduce the risk of variceal bleeding 3.
- JAK inhibitor therapies, such as ruxolitinib, have been shown to be effective in reducing splenomegaly and improving symptoms in patients with myelofibrosis 4, 5.
- The combination of ruxolitinib and hydroxyurea may be beneficial in patients with hyperproliferative forms of myelofibrosis, as it can improve clinical responses and increase ruxolitinib exposure 5.
Screening and Diagnosis
- Primary variceal screening may be beneficial in patients with myelofibrosis, as they are at risk of developing portal hypertension and variceal bleeding 2.
- Transjugular liver biopsy and radiological evaluation of the portal vascular system can be used to diagnose portal hypertension and establish the underlying cause 6.