title: Recurrent ischemic stroke in paroxysmal nocturnal hemoglobinuria: paroxysmal nocturnal hemoglobinuria or missed patent foramen ovale?
abstract: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired bone-marrow disorder characterized by hemolytic anemia, hemoglobinuria, and cytopenia. Most patients die from venous thrombotic events. Stroke is a common cause of morbidity and mortality in PNH and it is almost exclusively a result of cerebral venous thrombosis. Case reports of ischemic stroke complicating PNH have implicated a similar propensity for arterial events caused by the disease. We present a case of recurrent cerebral infarctions complicating PNH initially attributed to arterial thrombosis but ultimately determined to be a result of the disease and a concomitant patent foramen ovale identified only after repeated evaluations. This case emphasizes the pitfalls of diagnostic testing and the importance of a persistent search for a venous cause for cerebral embolic events in patients with hematologic diseases not classically known to involve the arterial system.
authors.affiliation: Department of Hematologic Oncology and Blood Disorders, Taussig Cancer Institute, Cleveland Clinic, Ohio 44195, USA. tiur@ccf.org
authors.forename: Ramon
authors.initials: R
authors.lastname: Tiu
authors.forename: Hussam
authors.initials: H
authors.lastname: Yacoub
authors.forename: Jaroslaw
authors.initials: J
authors.lastname: Maciejewski
authors.forename: Cathy A
authors.initials: CA
authors.lastname: Sila
mesh_terms: D002545:Brain Ischemia / Q000097:blood / Q000209:etiology* / Q000503:physiopathology; D015984:Causality; D002536:Cerebral Arteries / Q000473:pathology / Q000503:physiopathology; D002550:Cerebral Veins / Q000473:pathology / Q000503:physiopathology; D003937:Diagnosis, Differential; D003951:Diagnostic Errors / Q000517:prevention & control; D054092:Foramen Ovale, Patent / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D006801:Humans; D020244:Infarction, Middle Cerebral Artery / Q000097:blood / Q000209:etiology / Q000503:physiopathology; D020767:Intracranial Thrombosis / Q000097:blood / Q000209:etiology / Q000503:physiopathology; D018810:Magnetic Resonance Angiography; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D012008:Recurrence; D012680:Sensitivity and Specificity; D020521:Stroke / Q000097:blood / Q000209:etiology* / Q000503:physiopathology; D020246:Venous Thrombosis / Q000097:blood / Q000209:etiology / Q000503:physiopathology