Review Highlights Features, Treatment of Budd-Chiari Syndrome in MPN

Investigators reviewed available data on Budd-Chiari syndrome (BCS) in Philadelphia-negative myeloproliferative neoplasia (MPN) in a new review published in 2005. World Journal of Clinical Oncology.

Both conditions are rare, but BCS can occur in the MPN configuration.

“Individualized and unique counseling and multidisciplinary monitoring and treatment strategies are critical to achieving better outcomes in this patient population,” the study authors wrote. “People with MPN should be managed according to current guidelines to avoid developing BCS, but if diagnosed with BCS, the possibility of MPN diagnosis should be actively investigated. .”

MPN is a clonal disease of hematopoietic stem cells that causes excessive production of terminally differentiated myeloid cells. His MPN, Philadelphia-negative, includes polycythemia vera, essential thrombocytopenia, and primary myelofibrosis. All of these can lead to thrombotic complications such as BCS.

“BCS is a heterogeneous group of disorders characterized by obstruction of the hepatic venous outflow tract, ranging from the small hepatic vein, the three suprahepatic veins, to the junction of the inferior vena cava and the right atrium. across,” the authors explained.

Data on its condition are limited due to its extreme rarity. A recent meta-analysis estimated the prevalence to be 11 per million. These studies were mainly conducted in Asia and Europe. Another study in France found that the median age of patients with primary BCS was 47 years. About 70% of the patients in that study were women. Oral contraceptive use and pregnancy are gender-specific risk factors for BCS.

Although BCS is also characterized as thrombotic or non-thrombotic, thrombotic occlusion (primary BCS) is the most common cause of this condition. BCS can also be classified based on the site of obstruction and onset of disease pathology.

Inflammatory diseases such as thrombocytosis, thalassemia, paroxysmal nocturnal hemoglobinuria, MPN, pregnancy, oral contraceptive use, Behcet’s disease, celiac disease, and ulcerative colitis are all associated with primary BCS. However, MPN is the most common cause of BCS, with prevalence in the BCS setting ranging from 33% to 50%. A meta-analysis found polycythemia vera to be the most common MPN diagnosed in the BCS setting.

To the best of the authors’ knowledge, “there is no literature on variations in the histopathologic pattern of BCS secondary to MPN. Moreover, no similar prognostic grading system exists and is a potential area for further research.”

About 20% of people with BCS have few or no symptoms. Acute BCS has a short duration of onset of less than 1 month, while subacute BCS has a duration of onset ranging from 1 to 6 months.

Some data suggest geographic differences in the incidence of different types of BCS based on pathogenesis.

“Chronic symptoms are more prevalent in eastern regions, with time to onset ranging from 6 months to 30 years. In the western geographic region, acute symptoms occur more frequently,” the researchers explained. bottom.

For BCS due to MPN, anticoagulation serves as first-line treatment to relieve obstruction. Once the diagnosis is confirmed, antiplatelet therapy should also be started as soon as possible, the authors say.

However, only 15% to 20% of patients respond to anticoagulant therapy, so other interventions may be required in most cases. For acute or subacute BCS in MPN, one possible management option is angioplasty and stenting, but this intervention is usually reserved for symptomatic patients.


Găman M, Cozma M, Manan MR Budd-Chiari syndrome in other myeloproliferative neoplasms: a review of the literature. World J Clin Oncor. Published online March 24, 2023. doi:10.5306/wjco.v14.i3.99

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