Portal vein thrombosis as the first presentation of paroxysmal nocturnal hemoglobinuria

Abstract

paroxysmal nocturnal hemoglobinuria, portal vein thrombosis, anticoagulation therapy Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired clonal hematopoietic stem cell disorder, characterized by hemolytic anemia, bone marrow failure and thrombosis. Portal vein thrombosis (PVT) is relatively rare in patients with PNH. In this paper, we reported PVT as the first clinical presentation of PNH in a female patient. PVT related symptoms resolved after anticoagulation therapy.

Case report: Paroxysmal nocturnal hemoglobinuria presenting with hemorrhagic esophageal varices

“…PNH is a rare, acquired, potentially life-threatening hematologic disorder characterized by chronic intravascular hemolysis caused by uncontrolled activation of the terminal complement pathway (1). The mechanisms of thrombosis in PNH are still poorly understood; possible causes include the percentage of GPI protein-free granulocytes (PNH granulocytes >50%), endothelial cell damage, platelet activation because of the absence of CD59, and nitric oxide due to intravascular hemolysis, among others (7). Rother et al (8) pointed out that thromboembolism is mainly due to hemolysis; although the mechanism is not fully understood, hemolysis has been implicated in the initiation of platelet activation and aggregation.…”