Table 1

 Laboratory and clinical features of the patients

PatientAge/SexBleeding symptomsAssociated disordersPlatelet (×109/l)Bleeding timeRistocetinADP (2μM)ADP (10µM)Collagen (2µM)Epinephrine (10 µM)ATP release (2 µM ADP)ATP release (10 µM ADP)
CRF, chronic renal failure; EPO, erythropoietin (chronic anaemia); m, months; N, normal; ND, not done; y, years.
17y/MPresentCRF, EPO responsive anaemia, hepatomegaly23619 minNReduced primary aggregationMinimal improvementReduced primary aggregationReduced primary aggregationAbsentMinimal
21y 6m/MAbsentNone4069 minNAbsent secondary aggregationNNNND*ND*
31 y/FAbsentNone21725 minNAbsent secondary aggregationMinimal improvementNReduced secondary aggregationAbsentMinimal
43y 6m/MAbsentNone5109 min 30 sNNNNNNN
52y 6m/MAbsentNone23215 min 7 sNAbsent secondary aggregationMinimal improvementNAbsent secondary aggregationNDND
69y 8m/MPresentCRF25721 minNAbsent secondary aggregationNDNND*ND*ND*
75y 9m/MAbsentNone3004 min 30 sNNNNND*ND*ND*