Biological effects of a S/D-treated, very high purity, von Willebrand factor concentrate in five patients with severe von Willebrand disease
| Autor: | F. Meriane, M Goudemand, N. Djeha, C Mazurier, L. Zerhouni |
|---|---|
| Rok vydání: | 1993 |
| Předmět: |
congenital
hereditary and neonatal diseases and abnormalities medicine.medical_specialty biology medicine.diagnostic_test business.industry Shed blood Hematology General Medicine Plasma levels medicine.disease Gastroenterology Von Willebrand factor Blood product Bleeding time hemic and lymphatic diseases Internal medicine Immunology biology.protein Coagulopathy Von Willebrand disease Medicine business |
| Zdroj: | Blood Coagulation & Fibrinolysis. 4:1023-1029 |
| ISSN: | 0957-5235 |
| DOI: | 10.1097/00001721-199304060-00018 |
| Popis: | A highly purified von Willebrand factor (vWf) concentrate with low factor VIII (FVIII) content was tested to evaluate its biological effects in five patients with severe von Willebrand disease (vWd). The patients were infused with a single dose of this product corresponding to approximately 65 IU vWf:RCo and approximately 11 IU FVIII:C/kg and were followed up for 48 h. The plasma levels of FVIII and vWf and the multimeric pattern of vWf were determined before and 1, 3, 6, 12, 24 and 48 h after infusion. In all patients the recovery (93 +/- 14%) and half-life (13 +/- 1 h) of vWf were consistent and normal and sustained FVIII levels were attained. In four patients both the duration and the volume of shed blood were measured after one incision with a standardized template method of bleeding time (BT). The duration of bleeding was completely corrected in two out of the four patients and partially corrected in the other two while the volume of shed blood was completely corrected in three patients and partially corrected in the fourth. However, the time for correction of both BT parameters was variable between patients. These data indicate that the vWf concentrate can correct the quantitative defects related to plasma vWf deficiency in vWd patients whereas the effect on template BT is less reproducible. |
| Databáze: | OpenAIRE |
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