Von Willebrand disease: main characteristics and response to desmopressin.Study of 103 cases

Citation
Jm. Cesar et al., Von Willebrand disease: main characteristics and response to desmopressin.Study of 103 cases, MED CLIN, 111(16), 1998, pp. 601-603
Citations number
27
Categorie Soggetti
General & Internal Medicine
Journal title
MEDICINA CLINICA
ISSN journal
00257753 → ACNP
Volume
111
Issue
16
Year of publication
1998
Pages
601 - 603
Database
ISI
SICI code
0025-7753(19981114)111:16<601:VWDMCA>2.0.ZU;2-L
Abstract
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.