Storage Pool Disease Associated With Von Willebrand?

We evaluated a group of 11 patients (10 female, 1 male) complaining of minor bleeding symptoms that showed the pattern of storage pool disease (SPD) and besides, presented a lack of aggregation induced by ristocetin, which corrected with the addition of normal plasma (pool of 15 donors). Only two patients had familiar history of bleeding tendency. Bleeding time (Ivy) was prolonged in 63% of the patients and platelet retention (Pellem II) was decreased in 72% of them.

Three of the patients showed, apart from the SPD, a clear pattern of VW disease (low factor VIII: C and VIII: Ag). In four patients the levels of factor VIII: C and f, VIII:Ag were normal, but one of them presented a Ristocetin Cofactor (McFarlane) below normal, associated with a prolonged bleeding time and low adhesiveness. Three patients had low levels of VIII: C with normal levels of VIII:Ag. The last patient with border line levels of VIII: C and VIII:Ag, presented a low Ristocetin Cofactor. Thus, 5 of the 11 patients have undoubtful elements of VW. In the others this diagnosis could not be accomplished clearly.

As a conclusion, the presence of a characteristic aggregation pattern of SPD with absence of ristocetin induced aggregation, could suggest a variant of SPD or perhaps an association of both diseases