Acquired Von Willebrand Syndrome

Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder.

Désage et al. reported a case of AVWS due to a monoclonal gammopathy of undetermined significance, in which a transient but prolonged response to treatment by intravenous immunoglobulin (IVIG) was observed. The diagnosis was fortuitously made in a preoperative setting for neurosurgery, after biological exploration of an isolated prolonged activated partial thromboplastin time. AVWS was confirmed by an accelerated clearance of an infused plasma-derived von Willebrand factor (VWF) concentrate. High doses of IVIG were used to perform the neurosurgery. Fifty-four days after IVIG, the patient was still responding to treatment with normal levels of factor VIII and VWF 1).


Complications arising from antibiotic use are of interest to neurosurgeons because many neurosurgical patients are treated for an infection. In a report, Psarros et al. described three patients with spine disorders who developed coagulopathies after treatment with levofloxacin, an antibiotic commonly used by neurosurgical services. Three patients with spine disorders developed urinary tract infections (UTIs) for which they received a 3-day course of oral levofloxacin. Subsequently, they demonstrated prolonged prothrombin times and increased international normalized ratios. One of those patients later developed acquired von Willebrand syndrome during surgery. Coagulopathies were successfully corrected preoperatively with parenteral vitamin K. The patient with acquired von Willebrand syndrome required multiple transfusions. There seems to be an association between levofloxacin and coagulation abnormalities in neurosurgical patients treated for UTIs. Neurosurgical services prescribing this common antibiotic should be aware of this problem 2).


1)
Désage S, Le Quellec S, Karlin L, Lienhart A, Meunier S, Rugeri L. A Prolonged Treatment Response in Acquired Von Willebrand Syndrome. Hamostaseologie. 2019 Sep 23. doi: 10.1055/s-0039-1696960. [Epub ahead of print] PubMed PMID: 31546261.
2)
Psarros T, Trammell T, Morrill K, Giller C, Morgan H, Allen B. Abnormal coagulation studies associated with levofloxacin. Report of three cases. J Neurosurg. 2004 Apr;100(4):710-2. PubMed PMID: 15070128.
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