Major hemorrhage related to surgery in patients with type 2A con Willebrand disease

WOODS AI; MESCHENGIESER SS; SÁNCHEZ LUCEROS A; BLANCO AN; KEMPFER AC; LAZZARI MA

Buenos Aires

Congreso; XXIX International Congress of the World Federation of Hemophilia; 2010

World Federation of Hemophilia

Introduction: Type 2A variant of VWD is characterized by spontaneous bleeding; in many cases, severe. Objectives: Our aim was to identify predictive markers of surgical major hemorrhage in type 2A patients (pts). Material and Methods: We analyzed the presence of surgical major hemorrhages previous to diagnosis of VWD, and the clinical and laboratory parameters in 51 type 2A pts, with 76 surgeries. Pts were grouped according to the absence (A) or presence (B) of surgical major bleeding. Results: Thirty-eight surgeries (50%) in 28 pts (54.9%) showed major hemorrhages. Group A = 23 pts; females = 65.2%; surgeries = 29 (11 major, 9 minor, 6 adeno-tonsillectomies, 3 caesarean sections). Positive family history = 63.2%. Clinical symptoms: epistaxis = 60.8%; menorrhagia = 71.4%; hemorrhage post tooth extraction = 62.5%; postpartum = 28.5%; hematomas = 43.4; ‡3 bleeding sites = 47.8%; BS: males = 2.4 ± 2.3; ‡3 = 37.5%; females = 3.9 ± 2.15; ‡5 = 33.3%. Laboratory: FVIII = 60.2 ± 26.8; VWF:Ag = 81.3 ± 39.5; VWF:RCo = 21.3 ± 14.0; VWF:RCo/VWF:Ag = 0.26; prolonged BT = 71.4%. Group B = 28 pts; females = 65.2%; surgeries = 47 (13 major, 12 minor, 10 adeno-tonsillectomies, 12 caesarean sections). Positive family history = 84.2%. Clinical symptoms: epistaxis = 53.5%; menorrhagia = 64.7%; hemorrhage post tooth extraction = 70.5%; postpartum = 61.5%; hematomas = 53.5%; ‡3 bleeding sites = 53.6%; BS: males = 6.0 ± 3.5; ‡3 = 70%; females = 4.0 ± 2.2; ‡5 = 33.3%. Laboratory: FVIII = 55.5 ± 30.3; VWF:Ag = 76.9 ± 34.4; VWF:RCo = 22.9 ± 19.4; VWF:RCo/VWF:Ag = 0.297; prolonged BT = 70.3%. There were no differences in gender, positive family history, BS, and laboratory tests between patients with and without major hemorrhages related to surgery. A postpartum hemorrhage history was the most frequent clinical manifestation in pts with surgical major hemorrhage.Caesarean section and adeno-tonsillectomy showed the highest frequency of major hemorrhage (66% and 62% respectively). The BS and the number of bleeding sites were not predictors of surgical major hemorrhage. Conclusion: Laboratory tests proved ineffective in predicting major hemorrhages in type 2A VWD. The history of postpartum hemorrhage could predict surgical major hemorrhage.