Abstract
Background: Paroxysmal nocturnal hemoglobinuria is a rare acquired autoimmune disease relation to defect of hematopoietic stem, and is often associated with high rate of life-threatening venous thrombosis. Method: Lower extremity vein thrombosis is rarer than cerebral and hepatic veins in terms of thrombosis localization. Patients are exposed to intravascular hemolysis, smooth muscle dystonia, renal failure, arterial and pulmonary hypertension, recurrent infectious diseases and an increased risk of especially terrible thrombotic complications. The diagnosis is made by flow cytometry. Deficiency of the glycophosphatidylinositol - anchored complement regulatory proteins CD55 and CD59 accounts for the intravascular hemolysis that is the primary clinical manifestation of the disease. Besides, paroxysmal nocturnal hemoglobinuria is frequently associated with aplastic anemia or low-risk myelodysplasia and may be asymptomatic. Result: A patient, who developed thrombosis of the bilateral common iliac veins with the diagnosis of paroxysmal nocturnal hemoglobinuria, is described.
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