Hemorrhagic symptoms and bleeding risk in obligatory carriers of type 3 von Willebrand disease: an international, multicenter study
| Autor: | P. Petrini, A. Cappelletti, A. Srivastava, Alberto Tosetto, Francesco Baudo, J. S. Ungerstedt, M. Nishino, Jeroen Eikenboom, Augusto B. Federici, Stefan Lethagen, F. Rodeghiero, S. Linari, J. M. Lusher, Giancarlo Castaman |
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| Rok vydání: | 2006 |
| Předmět: |
Adult
Male Risk medicine.medical_specialty Pediatrics Heterozygote Blood transfusion Adolescent medicine.medical_treatment Population Hemorrhage Gastroenterology Von Willebrand factor Internal medicine Surveys and Questionnaires von Willebrand Factor Von Willebrand disease medicine Odds Ratio Humans Deamino Arginine Vasopressin education Desmopressin Child Aged education.field_of_study Factor VIII biology business.industry Infant Hematology Odds ratio Middle Aged medicine.disease Confidence interval von Willebrand Diseases Tranexamic Acid bleeding score carrier inherited bleeding disorders von Willebrand disease von Willebrand factor Case-Control Studies Child Preschool biology.protein Female business Settore MED/15 - Malattie del Sangue Tranexamic acid medicine.drug |
| Zdroj: | Journal of thrombosis and haemostasis : JTH. 4(10) |
| ISSN: | 1538-7933 |
| Popis: | Objectives: We undertook an international, multicenter study to describe the clinical picture and to estimate the bleeding risk in a group of obligatory carriers of type 3 von Willebrand disease (VWD). Patients and methods: Obligatory carriers (OC) of type 3 VWD were identified by the presence of offspring with type 3 VWD or by being an offspring of a type 3 patient. Normal controls were age- and sex-matched with the obligatory carriers. A physician-administered standardized questionnaire was used to evaluate hemorrhagic symptoms at presentation. A score system ranging from 0 (no symptom) to 3 (hospitalization, replacement therapy, blood transfusion) was used to quantitate bleeding manifestations. Odds ratios were computed for each symptom. Results: Ten centers participated to the study, enrolling a total of 35 type 3 VWD families, with 70 OC. A total of 215 normal controls and 42 OC for type 1 VWD were also included. About 40% of type 3 OC had at least one bleeding symptom compared to 23% of normal controls and 81.8% of type 1 OC (P < 0.0001 by chi-squared test), showing that type 3 OC clearly represent a distinct population from type 1 OC. The clinical situations associated with an increase of bleeding risk in type 3 OC were epistaxis [odds ratio 3.6; 90% confidence intervals (CI) 1.84-21.5], cutaneous bleeding (odds ratio 5.5; 90% CI 2.5-14.1) and postsurgical bleeding (odds ratio 16.3; 90% CI 4.5-59). The severity of bleeding score correlated with the degree of factor (F) VIII reduction in plasma. Conclusions: OC for type 3 VWD represent a distinctive population from type 1 OC. These patients, however, present with more frequent bleeding symptoms in comparison to normal controls, especially in case of significantly low FVIII. Desmopressin and/or tranexamic acid might be useful to prevent or treat bleeding in these cases. (Less) |
| Databáze: | OpenAIRE |
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