A double-blind trial of ticlopidine in sickle cell disease.

Baseline studies of 111Indium oxine labelled platelet life-span, platelet alpha-granule release products, beta-thromboglobulin (beta TG) and platelet factor 4 (PF4), and factor VIII related activities were performed on 9 asymptomatic patients with sickle cell disease, who were subsequently randomised in a prospective double-blind trial of ticlopidine (250 mg. b. d.) or placebo for one month and the investigations repeated. Control studies indicated that 5 of the 9 patients had shortened platelet survivals: mean beta TG (50.8 ng/ml) and PF4 (19.5 ng/ml), factor VIII:C (283.4 i.u./dl) and factor VIIIR:AG (168.7 u/dl) levels were raised. Ticlopidine treatment did not significantly improve platelet life-span or factor VIII levels, though it was associated with reduced values of beta TG and PF4. One patient taking ticlopidine developed an infarctive sickle crisis. Although ticlopidine blocked platelet activation, this alone did not improve platelet survival or prevent sickle crisis: in view of evidence of platelet activation in sickle cell disease, however, a longer trial of prophylactic antiplatelet drugs might be warranted.