Platelet Von Willebrand Factor in Hermansky-Pudlak Syndrome (American Journal of Hematology, Vol. 59, Issue 2) (1998)
Platelet Von Willebrand Factor in Hermansky-Pudlak Syndrome (American Journal of Hematology, Vol. 59, Issue 2) (1998)
*HPS, high molecular weight; vWF, von Willebrand factor; N.D., not done.
a
Bruising, bruising alone; minor, bruising, epistaxis, and other bleeding not requiring transfusion; major, bleeding requiring transfusion of red cells.
tivity were found in one third of the patients tested, no genic TG, as well as antigenic PF4 and vWF, other
relationship with clinical bleeding was observed. The ␣-granule proteins, to be within normal limits. However,
number of patients was not large, and a number of the we observed that platelet vWF activity was significantly
patients we studied were children who did not have lower in the HPS patients (P < 0.0001) when compared
bleeding times performed and required limited blood to the normals studied. Thirty-seven percent of the HPS
draws; several also had not encountered a significant patients had abnormally low levels of platelet vWF ac-
hemostatic challenge. tivity and an additional 19% had borderline values.
The number of ␣-granules present and the amount of We reported previously that platelet vWF activity and
the ␣-granule protein TG have been reported as normal antigen levels in type I von Willebrand disease correlate
in the HPS patients studied [2,8]. We also found anti- better with the bleeding time than do the plasma vWF
vWF in Hermansky-Pudlak Syndrome 119