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Hemorrhagic symptom...
Hemorrhagic symptoms and bleeding risk in obligatory carriers of type 3 von Willebrand disease: an international, multicenter study
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Castaman, G. (author)
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Rodeghiero, F. (author)
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Tosetto, A. (author)
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Cappelletti, A. (author)
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Baudo, F. (author)
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Eikenboom, J. C. J. (author)
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Federici, A. B. (author)
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- Lethagen, Stefan (author)
- Lund University,Lunds universitet,Institutionen för kliniska vetenskaper, Malmö,Medicinska fakulteten,Department of Clinical Sciences, Malmö,Faculty of Medicine
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Linari, S. (author)
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Lusher, J. (author)
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Nishino, M. (author)
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Petrini, P. (author)
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Srivastava, A. (author)
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- Ungerstedt, J. S. (author)
- Karolinska Institutet
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(creator_code:org_t)
- Elsevier BV, 2006
- 2006
- English.
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In: Journal of Thrombosis and Haemostasis. - : Elsevier BV. - 1538-7933 .- 1538-7836. ; 4:10, s. 2164-2169
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Abstract
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- 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.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Kardiologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Cardiac and Cardiovascular Systems (hsv//eng)
Keyword
- carrier
- inherited bleeding disorders
- von Willebrand
- von Willebrand factor
- disease
- bleeding score
Publication and Content Type
- art (subject category)
- ref (subject category)
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- By the author/editor
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Castaman, G.
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Rodeghiero, F.
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Tosetto, A.
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Cappelletti, A.
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Baudo, F.
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Eikenboom, J. C. ...
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show more...
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Federici, A. B.
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Lethagen, Stefan
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Linari, S.
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Lusher, J.
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Nishino, M.
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Petrini, P.
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Srivastava, A.
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Ungerstedt, J. S ...
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show less...
- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Cardiac and Card ...
- Articles in the publication
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Journal of Throm ...
- By the university
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Lund University
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Karolinska Institutet