Fatal haemorrhage due to extensive fragility of medium- and large-sized arteries and veins in a young patient with neurofibromatosis 1.
BACKGROUND
Neurofibromatosis type 1 (NF1) is one of the most frequently inherited diseases. Vascular manifestations have been reported, mostly concerning stenosis of the renal artery or spontaneous rupture of single arteries. Also, venous aneurysms have been reported.


METHODS
We present clinical and pathological findings of a young patient with NF1 with lethal bleeding due to spontaneous rupture of a lumbal and renal artery.


RESULTS
High fragility of several arteries as well as veins in the abdominal and pelvic arterial and venous system resulted in a destroyed vessel structure caused by invading neurogen fibres.


CONCLUSIONS
In rare cases, NF1 is associated with a severe systemic vasculopathy concerning the arterial and venous vascular system.
Hinsch
Nora
N
Institute of Pathology, Johann-Wolfgang-Goethe University Frankfurt, Frankfurt/Main, Germany. n.hinsch@em.uni-frankfurt.de
Kriener
Susanne
S
Ritter
Ralf-Gerhard
RG
Holzer
Katharina
K
D000328:Adult; D001158:Arteries / Q000473:pathology*; D002198:Capillary Fragility; D017809:Fatal Outcome; D005260:Female; D006470:Hemorrhage / Q000209:etiology*; D006801:Humans; D009456:Neurofibromatosis 1 / Q000150:complications*; D016491:Peripheral Vascular Diseases / Q000209:etiology*; D012422:Rupture, Spontaneous; D014680:Veins / Q000473:pathology*