Von Willebrand's disease is an inherited bleeding disorder witha prevalence as high as 1 to 2 percent in the general population,according to screening studies.1,2 In contrast, estimates basedon referral for symptoms of bleeding suggest a prevalence of30 to 100 cases per million, which is similar to the prevalenceof hemophilia A.1,2 The disease was first described in 1926by the Finnish pediatrician Erik von Willebrand, who used arowboat to make house calls to patients with the disease inthe Åland archipelago. The disease is caused by the quantitativedeficiency or dysfunction of von Willebrand factor, . . . [Full Text of this Article]
Classification
Laboratory Diagnosis
General Principles of Treatment
Autologous Replacement Therapy
Dosage and Routes of Administration
Clinical Efficacy
Response and Phenotypes
Adverse Effects
Allogeneic Replacement Therapy
Replacement Products
Dosage of Concentrate
Laboratory Monitoring
Antifibrinolytic Amino Acids
Reproductive Health
Menorrhagia
Management of Delivery
Alloantibodies
Acquired von Willebrand Syndrome
The Future
Conclusions
Source Information
From the Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and the Department of Internal Medicine and Dermatology, Istituto di Ricovero e Cura a Carattere Scientifico Maggiore Hospital and the University of Milan, Milan.
Address reprint requests to Dr. Mannucci at Via Pace 9, 20122 Milan, Italy, or at piermannuccio.mannucci@unimi.it.
Cuker, A., Connors, J. M., Katz, J. T., Levy, B. D., Loscalzo, J.
(2009). A Bloody Mystery. NEJM
361: 1887-1894
[Full Text]
Maquoi, I., Bonhomme, V., Born, J. D., Dresse, M.-F., Ronge-Collard, E., Minon, J.-M., Hans, P.
(2009). Perioperative Management of a Child with von Willebrand Disease Undergoing Surgical Repair of Craniosynostosis: Looking at Unusual Targets. Anesth. Analg.
109: 720-724
[Abstract][Full Text]
Rodeghiero, F., Castaman, G., Tosetto, A.
(2009). How I treat von Willebrand disease. Blood
114: 1158-1165
[Abstract][Full Text]
Lapecorella, M., Napoletano, M., Mariani, G., Mascaretti, G., Colageo, E.
(2009). Bleeding Management of Uterine Conization Using a Pasteurized FVIII/VWF Concentrate in a Woman Affected by Type 2A von Willebrand Disease. CLIN APPL THROMB HEMOST
15: 245-246
Sucker, C., Scharf, R. E., Zotz, R. B.
(2009). Use of Recombinant Factor VIIa in Inherited and Acquired von Willebrand Disease. CLIN APPL THROMB HEMOST
15: 27-31
[Abstract]
Czarnetzki, C., Elia, N., Lysakowski, C., Dumont, L., Landis, B. N., Giger, R., Dulguerov, P., Desmeules, J., Tramer, M. R.
(2008). Dexamethasone and Risk of Nausea and Vomiting and Postoperative Bleeding After Tonsillectomy in Children: A Randomized Trial. JAMA
300: 2621-2630
[Abstract][Full Text]
Davi, G., Patrono, C.
(2007). Platelet Activation and Atherothrombosis. NEJM
357: 2482-2494
[Full Text]
Federici, A. B., Castaman, G., Franchini, M., Morfini, M., Zanon, E., Coppola, A., Tagliaferri, A., Boeri, E., Mazzucconi, M. G., Rossetti, G., Mannucci, P. M.
(2007). Clinical use of Haemate(R) P in inherited von Willebrand's disease: a cohort study on 100 Italian patients. haematol
92: 944-951
[Abstract][Full Text]
van Schooten, C. J. M., Denis, C. V., Lisman, T., Eikenboom, J. C. J., Leebeek, F. W., Goudemand, J., Fressinaud, E., van den Berg, H. M., de Groot, P. G., Lenting, P. J.
(2007). Variations in glycosylation of von Willebrand factor with O-linked sialylated T antigen are associated with its plasma levels. Blood
109: 2430-2437
[Abstract][Full Text]
Ozgonenel, B., Rajpurkar, M., Lusher, J. M
(2007). How do you treat bleeding disorders with desmopressin?. Postgrad. Med. J.
83: 159-163
[Abstract][Full Text]
Michiels, J. J., van Vliet, H. H. D. M., Berneman, Z., Gadisseur, A., van der Planken, M., Schroyens, W., van der Velden, A., Budde, U.
(2007). Intravenous DDAVP and Factor VIII-von Willebrand Factor Concentrate for the Treatment and Prophylaxis of Bleedings in Patients With von Willebrand Disease Type 1, 2 and 3. CLIN APPL THROMB HEMOST
13: 14-34
[Abstract]
Crowther, M. A., Abshire, T. C.
(2007). Hemostasis and thrombosis. ASH-SAP
2007: 361-407
[Full Text]
Riddel, J. P. Jr., Aouizerat, B. E.
(2006). Genetics of von Willebrand disease type 1.. Biol Res Nurs
8: 147-156
[Abstract]
Pergolizzi, R. G., Jin, G., Chan, D., Pierre, L., Bussel, J., Ferris, B., Leopold, P. L., Crystal, R. G.
(2006). Correction of a murine model of von Willebrand disease by gene transfer. Blood
108: 862-869
[Abstract][Full Text]
Pruthi, R. K.
(2006). A Practical Approach to Genetic Testing for von Willebrand Disease. Mayo Clin Proc.
81: 679-691
[Abstract][Full Text]
Nakamura, F., Pudas, R., Heikkinen, O., Permi, P., Kilpelainen, I., Munday, A. D., Hartwig, J. H., Stossel, T. P., Ylanne, J.
(2006). The structure of the GPIb-filamin A complex. Blood
107: 1925-1932
[Abstract][Full Text]
James, A. H., Ragni, M. V., Picozzi, V. J.
(2006). Bleeding Disorders in Premenopausal Women: (Another) Public Health Crisis for Hematology?. ASH Education Book
2006: 474-485
[Abstract][Full Text]
Kashyap, A. S., Anand, K. P., Kashyap, S., Tanvetyanon, T., Mannucci, P.M.
(2004). Treatment of von Willebrand's Disease. NEJM
351: 2345-2346
[Full Text]