In platelets from 10 patients with severe homozygous-like VWD, VIII R:Ag was unmeasurable in seven extremely low in three when measured by a very sensitive IRMA; the VIII R:RCo necessary for normal platelet function was always unmeasurable. In 12 patients with 'classical' dominant VWD characterized by reduced plasma levels of VIII R:Ag and VIII R:RCo, these factor VIII-related properties were normal in platelets, and the mobility of VIII R:Ag on CIE was not different from that of normal platelets. In 7 patients showing a faster anodal mobility of plasma VIII R:Ag ('variant' VWD), the same abnormality was found in platelets. Moreover, platelet VIII R:Ag concentrations measured with EID were significantly higher than those of healthy individuals, whereas lower values were found with IRMA; VIII R:RCo was significantly decreased. These findings suggest that the severe form of VWD results from the marked or complete suppression of factor VIII synthesis fully expressed both in platelets and plasma; variant VWD appears to be related to the normal rate of synthesis of a functionally defective factor VIII molecule, whereas classical VWD is likely to be the expression of a defective transfer of the protein from the cellular compartments to plasma.
|Number of pages||9|
|Journal||The Journal of Laboratory and Clinical Medicine|
|Publication status||Published - 1978|
ASJC Scopus subject areas
- Pathology and Forensic Medicine