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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
 

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Catheter-directed thrombolysis and thrombectomy for the budd-chiari syndrome in paroxysmal nocturnal hemoglobinuria in three patients.

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem cell disorder characterized by hemolytic anemia, hemoglobinuria, bone marrow failure, and hypercoagulability. Thrombosis is the leading cause of mortality and occurs in one-half of PNH patients, with the hepatic veins being the most common site. Patients with hepatic vein thrombosis (Budd-Chiari syndrome) can present with abdominal pain, hepatomegaly, jaundice, and ascites. Prognosis is poor for these patients; death may occur from liver failure, vessel rupture, intestinal ischemia, infarction, necrosis, or sepsis. The authors report three consecutive cases of successful treatment with catheter-directed thrombolysis and thrombectomy directly in the hepatic veins in patients with PNH who developed acute hepatic vein thrombosis. This treatment represents a potential bridge toward more curative therapies such as allogeneic bone marrow transplant.[1]

References

  1. Catheter-directed thrombolysis and thrombectomy for the budd-chiari syndrome in paroxysmal nocturnal hemoglobinuria in three patients. Kuo, G.P., Brodsky, R.A., Kim, H.S. Journal of vascular and interventional radiology : JVIR. (2006) [Pubmed]
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