[Cerebral ischemia as first manifestation of paroxysmal nocturnal hemoglobinuria].

BACKGROUND

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal disease characterized by chronic intravascular hemolysis, cytopenia and increased tendency to thrombosis. Arterial thrombosis has been observed in patients with PNH but is uncommon compared with venous thrombosis. Cerebral arterial thrombosis has been anecdotally described as the first clinical manifestation of PNH.

METHODS

We present the case of a 48-yearold male who was admitted because of high-grade fever and infarction in the left middle cerebral artery territory. Other associated findings were mild anemia (hemoglobin level: 10.1 g/dl), renal ischemia and intra-abdominal venous thrombosis. Recurrent arterial and venous thromboembolic events were observed during his hospital stay. The diagnosis of PNH was confirmed by flow cytometric analysis.

CONCLUSIONS

PNH is a rare cause of cerebral ischemia and it should be considered in stroke patients with abnormal blood findings or marked prothrombotic tendency. We discuss the treatment guidelines and prevention of thrombotic complications.