Neurologische Komplikationen bei der paroxysmalen nächtlichen Hämoglobinurie

A patient with paroxysmal nocturnal haemoglobinuria (PNH) is presented. In the course of his disease he first developed a mesenterial thrombosis and shortly thereafter he had to be admitted with an intracranial thrombosis. He was treated by continuous infusion therapy of heparin, during which in spite of only a twofold increase of partial thromboplastin time a subdural and a psoas haematoma were observed. After recovery, the patient was discharged on low dose subcutaneous heparin therapy and had to be readmitted while on this treatment with a massive intracerebral haemorrhage. The possible mechanisms leading to these complications are discussed.