VENOUS THROMBOSIS IN A PATIENT WITH PAROXYSMAL NOCTURNAL HEMOGLOBINURIA. A CASE FROM PRACTICE, Svetomir Chakarov

Abstract: Paroxysmal Nocturnal Hemoglobinuria  (PNH), also called Marchiafava-Micheli syndrome or Strübing-Marchiafava anemia, is one of the acquired diseases associated with impaired bone marrow hematopoiesis (citation from radiopaedia https://radiopaedia.org/articles/paroxysmal-nocturnal-haemoglobinuria). It manifests with hemolytic anemia, thrombosis and bone marrow aplasia expressed to varying degrees. It manifests clinically in several ways, with thrombotic vascular incidents having important clinical significance. Approximately 40% of patients are involved in such incidents[1]. In this publication, I will present a patient who was diagnosed with paroxysmal nocturnal hemoglobinuria at the age of 21. At the age of 31, after two generalized epileptic seizures with tongue biting, loss of consciousness, no memory of what happened and weakness of the right limbs, he was hospitalized. After the performed imaging studies CT and MRI, thrombosis of the cortical vein on the left frontally was diagnosed, segmental areas of thrombosis of the superior sagittal venous sinus and sigmoid venous sinus bilaterally. During the hospital stay, a CT scan with CM of the abdomen was also performed due to hyperbilirubinemia, which revealed thrombosis of segmental branches of the left and right hepatic veins.

Keywords: Paroxysmal nocturnal hemoglobinuria, venous sinus thrombosis, hepatic vein thrombosis, computed tomography, magnetic resonance imaging.

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