BACKGROUND: to describe the main characteristics and response to desmopress
in infusion in 103 patients suffering from von Willebrand disease (vWD).
PATIENTS AND METHODS: The criteria for diagnosis were (except for type 2N)
the coexistence of von Willebrand factor ristocetin cofactor (VWF: RCo) act
ivity < 50 U/dl with bleeding disease or one of the following data: von Wil
lebrand factor antigen (vWF:Ag) activity < 50 U/dl, factor VIII (FVIII) act
ivity < 50 U/dl or the existence of a increased bleeding time (BT). Multime
ric studies of vWF were performed in 51 cases and ristocetin induced platel
et aggregation (RIPA) was also performed.
RESULTS: Spontaneous bleeding was found in 36 patients, while in 18 cases t
he diagnosis was done after surgical bleeding. Thirteen patients (6 present
ing with mild bleeding) were studied for abnormalities in the routine prean
estesic tests. Other 22 patients were diagnosed with vWD by familial studie
s. There were 3 patients with type 2B, 1 case with type 2N and other patien
t with type 3. BT was found increased in 26 out of 58 patients. The activit
ies of vWF:CoR and vWF:Ag were 38,4 (9.4) U/dl and 45.8 (23.2) U/dl, respec
tively, while the activity of FVIII was 49.9 (20.8) U/dl. Prophylactic DDAV
P (desmopressin) was infused in 32 patients. After 1 h, basal activities of
vWF:CoR and vWF:Ag were increased by 3.1 (3.2) and 3.4 (3.1) times, respec
tively, and maintained for 3 h. FVIII activity increased 3.6 (2.3) times th
e basal levels decreasing after 3 h (2.9 [2.1]; p < 0.01). The BT was corre
cted in 8 out of ten patients.
CONCLUSIONS: vWD is a major cause of surgical bleeding. Preanestesic anamne
sis and coagulation tests can be useful to identify vWD. Many patients with
vWD have normal BT. A failure in the response to desmopressin infusion is
unusual.