Early transfusion of factor VIII/von Willebrand factor concentrates seems to be effective in the treatment of gatrointestinal bleeding in patients with von Willebrand type III disease

Citation
E. Zanon et al., Early transfusion of factor VIII/von Willebrand factor concentrates seems to be effective in the treatment of gatrointestinal bleeding in patients with von Willebrand type III disease, HAEMOPHILIA, 7(5), 2001, pp. 500-503
Citations number
14
Categorie Soggetti
Hematology
Journal title
HAEMOPHILIA
ISSN journal
13518216 → ACNP
Volume
7
Issue
5
Year of publication
2001
Pages
500 - 503
Database
ISI
SICI code
1351-8216(200109)7:5<500:ETOFVW>2.0.ZU;2-F
Abstract
The association between gastrointestinal angiodysplasia and von Willebrand disease was reported 30 years ago. The clinical course of patients with von Willebrand disease and angiodysplasia is characterized by numerous admissi ons to hospital for gastrointestinal bleeding necessitating transfusion wit h packed red cells, factor VIII and plasma. The management of these patient s is problematic. Numerous treatments for the gastrointestinal bleeding hav e been proposed: surgery, electrocoagulation, laser photocoagulation, scler otherapy, arteriography with embolization, immunoglobulins, oestrogens, and octreotide, but no treatment modality has been successful in all cases. We report a 66-year-old-female with small bowel angiodysplasia and von Willeb rand type III disease in whom prompt administration of factor VIII/vWF conc entrates was effective. Education of patients to recognize minimal gastroin testinal bleeding manifestations, periodical clinical visits and early infu sion of factor VIII/vWF seems to be fundamental for the success of this the rapy. A longer follow-up and the Study of other patients are needed to conf irm our observation.