Summary
The Gray platelet syndrome is a rare disorder characterised by the absence of platelet a-granules and their contents. We describe a new patient and the effects of infusions of l-deainino-8-aiginine vasopressin (DDAVP). The patient had a prolonged skin bleeding time and his platelets had reduced numbers of a-granules, increased vacuolation and reduced retention on glass beads. Flatelet von Willebrand factor antigen (vWf:Ag) was undetectable and levels of platelet fibrinogen, p-thioniboglobulin, platelet factor 4 and thrombospondin were reduced. All tests of plasma coagulation factors were normal, including Factor VIII (F. VIII: C), vWf: Ag, ristocetin cofactor (R: CoF) and botrocetin cofactor. Platelet ATP, ADP, platelet albumin, surface membrane glycoproteins and 14C-serotonin uptake were also normal. Infusions of DDAVP increased plasma F.VIII:C, vWf:Ag and R.CoF and sliuitened the bleeding time un two occasions. This suggests that DDAVP shortens the bleeding time by releasing vWf: Ag and/or other proteins from cellular storage sites other than the platelet.
Keywords
Gray platelets - α-granules - von Willebrand factor - 1-deamino-8-arginine vasopressin - Bleeding time
