Paroxysmal nocturnal hemoglobinuria with sagittal vein thrombosis as the initial presentation

Introduction: Paroxysmal nocturnal hemoglobinuria is a rare disease that presents with variety of symptoms including hematuria, thrombosis, and other non-specific symptoms. Venous thrombosis not uncommon in paroxysmal (PNH). However, thromboembolic event as the presenting symptom PNH can be life-threatening, when affects sagittal veins brain. Case Report: A 28-year-old female presented to facility left-sided hemiparesis. Initial workup revealed right-sided subacute hemorrhagic infarct parietal lobe, magnetic resonance imaging (MRI) venography demonstrated extensive venous involving sagittal, straight, transverse sinuses, secondary edema involved hemisphere. Thrombotic events may affect up 50% patients account for at least one-third mortality PNH. Although presentation variable, cerebral sinus first thrombotic along absence hematuria makes this case unique. High suspicion differential diagnosis should considered young these symptoms, even hematuria. Ravulizumab has significantly reduced events, therapies immediately commenced upon high thrombosis. Allogeneic hematopoietic stem cell transplantation offers only curative approach date. Conclusion: very life-threatening disease. Hematuria always present Complement inhibitor therapy started confirmation decrease future events. need allogeneic transplantation, which available today.