Portal Vein Thrombosis in Paroxysmal Nocturnal Haemoglobinuria

Abstract

A 28-year-old man was hospitalized with nausea, vomiting, abdominal pain and low-grade fever. He had a 6-month history of paroxysmal nocturnal haemoglobinuria (PNH), and laboratory data showed anaemia and liver dysfunction. An abdominal ultrasonography showed ascites and portal vein thrombosis. After receiving antithrombotic treatment, the portal vein thrombosis did not extend. Portal vein thrombosis is very rare but should be considered when we encounter liver dysfunction associated with PNH as well as hepatic vein thrombosis. Ultrasonography is very useful in detecting portal vein thrombosis and facilitating early diagnosis. Warfarin is very effective in preventing exacerbation of portal vein thrombosis in PNH.

The hypercoagulable state in thalassemia

“…95,96 Other congenital hemolytic anemias also carry an increased risk for thromboembolic events. 59 PNH is reported to be associated with an increased tendency for DVT and portal vein thrombosis, 97,98 and frequent thrombotic episodes were reported in 9 patients with hereditary stomatocytosis. Data on abnormal hemostatic parameters in these disorders are currently not available.…”

The Presence of Paroxysmal Nocturnal Hemoglobinuria in Patients with Idiopathic Chronic Renal Failure

“…The most important complication that develops in the course of PNH is thrombotic events, which affect the abdominal veins. Thrombotic events occur frequently in hepatic, splenic, mesenteric, renal, and portal veins (18). None of the patients in our study had a history of arterial or venous thrombosis.…”

Portal hypertension resulted from paroxysmal nocturnal hemoglobinuria: a case report and review of literature

“…Supportive care with folic acid, blood transfusions, anticoagulation, antithymocyte globulin, cyclosporine and the occasional use of glucocorticosteroid are of treatment therapeutic regimens for most patients with PNH. The cornerstone of medical management is anticoagulation therapy following an acute thrombotic event to prevent further extension of venous thrombosis [9]. Administration of heparin with a thrombolytic is the treatment of choice in acute venous thrombosis.…”