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A Journal on Surgery
Indexed/Abstracted in: EMBASE, Scopus, Emerging Sources Citation Index
Chirurgia 2012 April;25(2):129-31
Symptomatic lumbar intratumoral schwannoma hemorrhage as the first manifestation of hemophilia A: case report
Fargen K. M., Murad G. J.
Department of Neurosurgery, University of Florida College of Medicine, Gainesville, FL, USA
Hemophilia A is a genetic bleeding disorder due to insufficient or absent factor VIII. Although intracranial hemorrhage occurs in approximately 3-10% of hemophilia patients, intratumoral hemorrhage in this population is exceedingly rare. Intratumoral schwannoma hemorrhage in this population has never before been reported. A previously-healthy 22 year old male presented with acute onset paraparesis and urinary dysfunction. Imaging demonstrated a large intradural hemorrhagic mass at L1. Intraoperatively, a schwannoma was identified, along with subdural and subarachnoid hemorrhage, and gross total resection was performed. Post-operatively, the patient developed a large wound hematoma resulting in dehiscence requiring wound revision. A bleeding workup revealed hemophilia A with markedly diminished Factor VIII activity (7%, reference range 60-200%). Recombinant factor VIII was given at time of wound revision without further complications.
Conclusion. Intratumoral hemorrhages in schwannomas have been reported in mild trauma or anticoagulated patients but never before in the setting of hemophilia. Surgical prophylaxis with recombinant factor VIII may normalize the risk of bleeding complications in patients with hemophilia A. Spontaneous intratumoral hemorrhage in a schwannoma due to hemophilia has not been previously reported and the rarity of spontaneous hemorrhage should elicit a thorough review of risk factors and history of a bleeding diathesis in patients with schwannomas. Discovery of hemophilia after intratumoral hemorrhage and wound hematoma led to adequate treatment at the next surgery, potentially preventing further bleeding complications.