Portal vein thrombosis as the first presentation of paroxysmal nocturnal hemoglobinuria

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.