Assay Patient value Normal range Hemoglobin 7.7 mmol/l 7.3-9.7 mmol/l Hematocrit 0.38 0.34-0.46 Thrombocytes 243×109/l 120×109-350×109/l Mean platelet volume 7.6 fl 7.5-11.5 fl Ristocetin cofactor activity (VWF) 165% 50-185% VWF multimers Normal NAa Collagen binding assay 144% 64-155% Factor-VIII activity 180% 60-150% ADP aggregation Normal NAa Collagen aggregation Normal NAa Arachidonic acid aggregation Normal NAa PFA (collagen-epinefrin) 97 sec <170 sec PFA (collagen-ADP) 74 sec <120 sec Ristocetin agglutinationb Normal NAa GP53 (CD63) Normal NAa GPIIbIIIa (PAC-1) Normal NAa P-selectin (CD62p) Normal NAa aNA stands for not applicable, no reference values are known, the patient sample was compared to the normal control samples. bThe platelet aggregation was measured using platelet rich plasma in the presence of 0.5, 0.6, 0.7,0.8, 0.9 and 1.52 mg/ml of ristocetin.

Table 2: Hematological analysis of patient carrying GPIbα sequence variation.