Hereditary/Acquired Bleeding Disorders

This case study describes a 48-year-old female with polycythemia vera (PV, a chronic myeloproliferative neoplasm characterized by thrombosis) diagnosed in China 15 years prior. At the authors' clinic, the patient presented with right eye pain following accidental low-impact head trauma. For pain relief, she took preparations of Chinese herbs as well as aspirin. CT of the head showed right-sided subdural hematoma. An aneurysm arising from a right middle cerebral atery (MCA) branch was revealed by direct angiographic imaging. Von Willebrand factor (VWF) activity was reduced to 26%. Three years prior to presentation, a ristocetin-induced platelet aggregation assay was performed as part of an evaluation of heavy menstrual bleeding; this assay revealed decreased VWF activity. Prior to undergoing craniotomy, the patient was given 1-deamino-8-D-arginine vasopressin (DDAVP). The aneurysm-like structure was found to be a small grape-like structure of the fibrinous part of the subdural membrane that had formed from the subdural hematoma. In discussing this study, the authors wrote, "Acquired von Willebrand syndrome (AVWS) is an important risk factor for bleeding in PV. DDAVP may be useful to increase levels of [VWF] and decrease the risk of bleeding perioperatively. Exogenous substances such as ginseng should be investigated as possible contributors to bleeding tendency and discontinued."