Some hospital laboratories and referral laboratories reported low vWF values and poor platelet response to ristocetin while others reported normal values. She was not tested for other coagulation factor deficiencies or malfunction. Cross linked fibrin clots are insoluble in urea. The patient's plasma was assayed for factor XIIIa transglutaminase activity by dansylcadaverine incorporation into a casein substrate. There was practically no shift in wavelength of emitted fluorescent light indicating that enzyme activity could not be generated in the patient's plasma.
Platelet numbers are within normal range.
Coagulation pathways are intact. No evidence of von Willebrand Disorder. Fibrinogen is quantitatively and qualitatively within normal range. Immediate clot lysis. Immediate solubility in 4 molar urea.