Paroxysmal nocturnal hemoglobinuria case presenting as cerebral venous sinus thrombosis
Keywords:Paroxysmal nocturnal hemoglobinuria, Cavernous venous sinus thrombosis, Eculizumab
Venous thromboembolism (VTE) is the leading cause of morbidity and mortality in paroxysmal nocturnal hemoglobinuria (PNH). Between 29% and 44% of PNH patients experience a clinically evident VTE that affects the liver, brain, gut or kidney. Cases of VTE have been reported in all major organs except the spinal cord and bone marrow. Cavernous venous sinus thrombosis (CVST) is rare, but it has been reported previously to occur late in the course of PNH. Here we describe a case of CVST in a 34-year-old female admitted to our hospital with severe left sided temporal headache, double vision and malaise as an initial manifestation of PNH. This is a rare case of CVST as a presenting manifestation of PNH. This case along with two other recent reports of CVST accompanied by haemolytic anaemia in patients with PNH encourages increased vigilance for PNH in patients without an associated thrombophilic condition.
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Copyright (c) 2018 Hilmi Erdem Sümbül, Mahmut Büyükşimşek, Merve Saraçoğlu Sümbül, Birol Güvenç
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