Use of rFVIIa in Four Patients With Glanzmann Thrombasthenia
| Patient No. . | Age (yr)/Sex . | Bleeding Episodes (n) . | Surgery (n) . | Clinical Efficacy* . | ||||||
|---|---|---|---|---|---|---|---|---|---|---|
| Nose . | Oropharynx . | GI . | Hematoma . | Excellent . | Good . | Recurrence . | Ineffective . | |||
| 1† | 6/M | — | 1 | 1 | — | 1 | 3/3 | — | — | — |
| 2†,‡ | 0.8/F | 10 | 6 | 1 | 1 | — | 12/18 | 4/18 | 21-153/18 | — |
| 3 | 3/F | 1 | — | 1 | — | — | — | 1/2 | — | 11-155/2 |
| 4 | 2/F | 2 | — | — | — | — | — | 2/2 | — | — |
| Patient No. . | Age (yr)/Sex . | Bleeding Episodes (n) . | Surgery (n) . | Clinical Efficacy* . | ||||||
|---|---|---|---|---|---|---|---|---|---|---|
| Nose . | Oropharynx . | GI . | Hematoma . | Excellent . | Good . | Recurrence . | Ineffective . | |||
| 1† | 6/M | — | 1 | 1 | — | 1 | 3/3 | — | — | — |
| 2†,‡ | 0.8/F | 10 | 6 | 1 | 1 | — | 12/18 | 4/18 | 21-153/18 | — |
| 3 | 3/F | 1 | — | 1 | — | — | — | 1/2 | — | 11-155/2 |
| 4 | 2/F | 2 | — | — | — | — | — | 2/2 | — | — |
Response according to number of hours to stop bleeding after start of rFVIIa—Excellent: ≤6 hours without recurrence; Good: >6 ≤ 24 hours without recurrence; Recurrence: ≤24 hours but with recurrence of bleeding; Ineffective: >24 hours and/or requiring platelet transfusion to stop bleeding.
Patients 1 and 2 are siblings.
Patient 2 had alloantibodies against GPIIb/IIIa.
Bleeds stopped with no further recurrences after additional doses of rFVIIa.
Bleed stopped after platelet transfusion.