Platelet-related laboratory results
| . | Platelet aggregation . | Platelet lysate . | Plasma . | Platelet plasma membrane at rest . | Platelet plasma membrane after thrombin receptor-mediated stimulation . | |||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Family no. (patient no.) . | ADP . | Epinephrine . | Arachidonic acid . | Collagen . | Thrombin . | PF4 . | Fibrinogen . | Fibronectin . | Platelet vWf antigen (%) . | Platelet vWf activity (%) . | vWf antigen (IU/dL) . | vWf activity (IU/dL) . | P-selectin (%)* . | Fibrinogen receptor (%)* . | TSP-1 (%)* . | P-selectin (%)* . | Fibrinogen receptor (%)* . | TSP-1 (%)* . |
| 1 (V-20) | Normal | Normal | Normal | Normal | Normal | - | 45 ng/106 cells (Normal:70) | 0.34 ng/106 cells | < 3 | < 3 | - | - | - | - | - | - | - | - |
| 1 (V-21) | Normal | Normal | Normal | Normal | - | 2.3 ng/106 cells (Normal:17-23) | 33 ng/106 cells (Normal:70) | - | < 3 | < 3 | - | - | - | - | - | - | - | - |
| 2 (VII-1) | Normal | - | - | Normal | - | - | - | - | - | - | - | - | - | - | - | - | - | - |
| 2 (VII-2) | Normal | - | - | Normal | - | - | - | - | - | - | - | - | - | - | - | - | - | - |
| 3 (IV-1) | Normal17 | No aggregation17 | Normal | No aggregation17 | - | - | 1.25 μg/L08 cells17 (Normal:28) | - | 417 | - | - | - | - | - | - | 50 | - | - |
| 4 (IV-1) | Reduced | Reduced | - | Reduced | - | - | - | - | 4 | < 3 | 116 | 123 | - | - | - | - | - | - |
| 4 (IV-2) | Reduced | - | - | Reduced | - | - | - | - | 4 | < 3 | 96 | 99 | - | - | - | - | - | - |
| 7 (II-2) | Normal6 | Normal6 | - | Reduced to ∼30%6 | - | 66 ng/108 cells6 (Normal: 2040 ± 320) | - | - | - | - | - | - | 121 | - | 105 | 12 | - | 7 |
| 7 (II-3) | - | - | - | - | - | 145 ng/108 cells6 (Normal: 2040 ± 320) | - | - | - | - | - | - | 213 | - | 144 | 17 | - | 7 |
| 8 (II-1) | Normal8 | Normal8 | - | Normal8 | - | 17 ng/108 cells8 (Normal: 1200-3000) | Undetectable8 | - | - | - | - | - | - | - | - | - | - | - |
| 11 (II-1) | Normal | Normal | Normal | Normal | Normal | - | - | - | - | - | - | - | 173 | 160 | - | 68 | 76 | - |
| 13 (II-1) | Normal | Normal | Normal | Normal | Normal | - | - | - | - | - | - | - | 72 | 128 | - | 60 | 173 | - |
| 14 (II-1) | Normal | Normal | Normal | Normal | Normal | - | - | - | < 3 | < 3 | 80 | 63 | - | - | - | - | - | - |
| Normal | Normal | Normal | Normal | Normal | Normal | Varies as above | Varies as above | 1.4-1.8 | 16-55 | 44-115 | 41-132 | 48-144 | 100 | 100 | 100 | 100 | 100 | 100 |
| . | Platelet aggregation . | Platelet lysate . | Plasma . | Platelet plasma membrane at rest . | Platelet plasma membrane after thrombin receptor-mediated stimulation . | |||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Family no. (patient no.) . | ADP . | Epinephrine . | Arachidonic acid . | Collagen . | Thrombin . | PF4 . | Fibrinogen . | Fibronectin . | Platelet vWf antigen (%) . | Platelet vWf activity (%) . | vWf antigen (IU/dL) . | vWf activity (IU/dL) . | P-selectin (%)* . | Fibrinogen receptor (%)* . | TSP-1 (%)* . | P-selectin (%)* . | Fibrinogen receptor (%)* . | TSP-1 (%)* . |
| 1 (V-20) | Normal | Normal | Normal | Normal | Normal | - | 45 ng/106 cells (Normal:70) | 0.34 ng/106 cells | < 3 | < 3 | - | - | - | - | - | - | - | - |
| 1 (V-21) | Normal | Normal | Normal | Normal | - | 2.3 ng/106 cells (Normal:17-23) | 33 ng/106 cells (Normal:70) | - | < 3 | < 3 | - | - | - | - | - | - | - | - |
| 2 (VII-1) | Normal | - | - | Normal | - | - | - | - | - | - | - | - | - | - | - | - | - | - |
| 2 (VII-2) | Normal | - | - | Normal | - | - | - | - | - | - | - | - | - | - | - | - | - | - |
| 3 (IV-1) | Normal17 | No aggregation17 | Normal | No aggregation17 | - | - | 1.25 μg/L08 cells17 (Normal:28) | - | 417 | - | - | - | - | - | - | 50 | - | - |
| 4 (IV-1) | Reduced | Reduced | - | Reduced | - | - | - | - | 4 | < 3 | 116 | 123 | - | - | - | - | - | - |
| 4 (IV-2) | Reduced | - | - | Reduced | - | - | - | - | 4 | < 3 | 96 | 99 | - | - | - | - | - | - |
| 7 (II-2) | Normal6 | Normal6 | - | Reduced to ∼30%6 | - | 66 ng/108 cells6 (Normal: 2040 ± 320) | - | - | - | - | - | - | 121 | - | 105 | 12 | - | 7 |
| 7 (II-3) | - | - | - | - | - | 145 ng/108 cells6 (Normal: 2040 ± 320) | - | - | - | - | - | - | 213 | - | 144 | 17 | - | 7 |
| 8 (II-1) | Normal8 | Normal8 | - | Normal8 | - | 17 ng/108 cells8 (Normal: 1200-3000) | Undetectable8 | - | - | - | - | - | - | - | - | - | - | - |
| 11 (II-1) | Normal | Normal | Normal | Normal | Normal | - | - | - | - | - | - | - | 173 | 160 | - | 68 | 76 | - |
| 13 (II-1) | Normal | Normal | Normal | Normal | Normal | - | - | - | - | - | - | - | 72 | 128 | - | 60 | 173 | - |
| 14 (II-1) | Normal | Normal | Normal | Normal | Normal | - | - | - | < 3 | < 3 | 80 | 63 | - | - | - | - | - | - |
| Normal | Normal | Normal | Normal | Normal | Normal | Varies as above | Varies as above | 1.4-1.8 | 16-55 | 44-115 | 41-132 | 48-144 | 100 | 100 | 100 | 100 | 100 | 100 |
Percentage of geometric fluorescence mean where 100% is the value obtained for the normal control subject.
ADP indicates adenosine diphosphate; PF4, platelet factor 4; vWf, von Willebrand factor; TSP-1, thrombospondin-1; and -, not tested.