[Intradural hematoma of the foramen magnum associated with factor XIII deficiency].
A 50-year-old woman, with a history of IgG monoclonal gammapathy, presented with meningitis and intradural hematoma of the foramen magnum associated with factor XIII deficiency. The patient died postoperatively of diffuse haemorrhage. Inhibitors to factor XIII are extremely rare and are congenital or acquired. Patients with factor XIII inhibitor can experience severe bleeding, and many died of cerebral haemorrhage. The role of this defect is discussed. We recommend an extensive investigation of haemostasis for patients with both episode of haemorrhagic disorder and monoclonal gammapathy.
Donnet
A
A
Service de Neurochirurgie, CHU Timone, Marseille.
Trefouret
S
S
Alessi
M C
MC
Harlé
J R
JR
Graziani
N
N
Grisoli
F
F
D005177:Factor XIII Deficiency / Q000150:complications*; D017809:Fatal Outcome; D005260:Female; D005539:Foramen Magnum / Q000098:blood supply; D006406:Hematoma / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008580:Meningism / Q000209:etiology; D008875:Middle Aged; D010265:Paraproteinemias / Q000150:complications*; D013118:Spinal Cord Diseases / Q000175:diagnosis / Q000209:etiology*; D014057:Tomography, X-Ray Computed
Hématome intradural du foramen magnum associé à un déficit en facteur XIII.