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
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
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.