Platelet-associated data
. | WASP . | . | . | |
|---|---|---|---|---|
. | Positive . | Negative . | P . | |
| No. patients | 27 | 23 | — | |
| Major bleeding problems | ||||
| Intracranial hemorrhage, n (%) | 5 (18.5) | 6 (26.1) | .52† | |
| Events/patient-year | 0.015 | 0.042 | .06† | |
| Age, y, mean | 11.0 | 3.2 | .10† | |
| Age range, y | 2-28 | 1-7 | ||
| Intestinal bleeding, n (%) | 3 (11.1) | 15 (65.2) | < .0001 | |
| Events/patient-year | 0.01 | 0.13 | < .0001 | |
| Death due to hemorrhage, n (%) | 1 (3.7) | 4 (17.4) | .11† | |
| Platelet count on first visit, × 109/L | ||||
| Mean ± SD (n) | 36 ± 31 (13) | 37 ± 19 (14) | .94† | |
| Range | 0.6-11.6 | 1.0-7.0 | — | |
| Mean platelet volume, fL | ||||
| Mean ± SD (n) | 6.0 ± 1.4 (18) | 6.2 ± 1.7 (15) | .63† | |
| Range | 4.0-8.8 | 4.0-10.5 | — | |
| BM megakaryocyte* | ||||
| Mean ± SD (n) | 97.6 ± 97.0 (13) | 65.2 ± 108.5 (15) | .42† | |
| Range, /μL | 0-320 | 0-385 | — | |
| Normal or increased, n (%) | 7 (54) | 4 (27) | — | |
| Decreased, n (%) | 5 (38) | 4 (27) | — | |
| Undetectable, n (%) | 1 (8) | 7 (46) | — | |
| PAlgG | ||||
| Mean ± SD, ng/107 platelets (n) | 434.6 ± 979.7 (20) | 272.8 ± 228.0 (14) | .55† | |
| Range, ng/107 platelets | 12.5-4157 | 2.5-789 | — | |
| No. PAlgG-positive patients | 20 | 13 | — | |
. | WASP . | . | . | |
|---|---|---|---|---|
. | Positive . | Negative . | P . | |
| No. patients | 27 | 23 | — | |
| Major bleeding problems | ||||
| Intracranial hemorrhage, n (%) | 5 (18.5) | 6 (26.1) | .52† | |
| Events/patient-year | 0.015 | 0.042 | .06† | |
| Age, y, mean | 11.0 | 3.2 | .10† | |
| Age range, y | 2-28 | 1-7 | ||
| Intestinal bleeding, n (%) | 3 (11.1) | 15 (65.2) | < .0001 | |
| Events/patient-year | 0.01 | 0.13 | < .0001 | |
| Death due to hemorrhage, n (%) | 1 (3.7) | 4 (17.4) | .11† | |
| Platelet count on first visit, × 109/L | ||||
| Mean ± SD (n) | 36 ± 31 (13) | 37 ± 19 (14) | .94† | |
| Range | 0.6-11.6 | 1.0-7.0 | — | |
| Mean platelet volume, fL | ||||
| Mean ± SD (n) | 6.0 ± 1.4 (18) | 6.2 ± 1.7 (15) | .63† | |
| Range | 4.0-8.8 | 4.0-10.5 | — | |
| BM megakaryocyte* | ||||
| Mean ± SD (n) | 97.6 ± 97.0 (13) | 65.2 ± 108.5 (15) | .42† | |
| Range, /μL | 0-320 | 0-385 | — | |
| Normal or increased, n (%) | 7 (54) | 4 (27) | — | |
| Decreased, n (%) | 5 (38) | 4 (27) | — | |
| Undetectable, n (%) | 1 (8) | 7 (46) | — | |
| PAlgG | ||||
| Mean ± SD, ng/107 platelets (n) | 434.6 ± 979.7 (20) | 272.8 ± 228.0 (14) | .55† | |
| Range, ng/107 platelets | 12.5-4157 | 2.5-789 | — | |
| No. PAlgG-positive patients | 20 | 13 | — | |
WASP-positive numbers represent WASP detectable by Western blot. WASP-negative numbers represent WASP found absent or truncated by Western blot. PAlgG indicates platelet-associated lgG; and —, not applicable.
BM megakaryocyte indicates number of bone marrow megakaryocytes per microliter BM aspirate. For BM megakaryocyte subrows, normal or increased indicates more than 50/μL; decreased, 20-50/μL; and undetectable, less than 20/μL.
Not significant.