Neurologische Komplikationen bei der paroxysmalen nächtlichen Hämoglobinurie

Summary

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.

Zusammenfassung

Es wird ein Patient mit einer paroxysmalen nächtlichen Hämoglobinurie (PNH), einer seltenen Krankheit der Stammzellen des Knochenmarks beschrieben, der kurz hintereinander zunächst eine intrakranielle Thrombose, danach eine intrazerebrale Massenblutung erlitt. Die Pathogenese dieser Komplikation und die möglichen therapeutischen Konsequenzen werden diskutiert.