title: Monocentric study of Willebrand's disease in Tunisia: assets and difficulties.
abstract: BACKGROUND
  Von Willebrand's disease (VWD) is the most commonly inherited bleeding disorder. It is characterized by clinical, biological and molecular heterogeneity. In certain types of the disease, diagnosis can be difficult.
  
  
  OBJECTIVE
  We report the clinico-biological characteristics of VWD's patients and analyze diagnosis difficulties.
  
  
  METHODS
  33 cases were diagnosed in the laboratoryfrom February to May, 2011. Screening hemostasis included the measuring of
  C, VWF: Ag and VWF: RCo. Blood cell count and blood group were performed in all cases.
  
  
  RESULTS
  Mean age at diagnosis is 13 years [10 months -43 years]. The sex ratio M/F is 0.5. The patients are classified type 3 VWD in 52% of the cases, type 2 VWD in 30 % of the cases and type 1 VWD in 18 % of the cases. The diagnosis of type 2B VWD suspected in combination of the ratio VWF:RCo / VWF: Ag <0,7 and thrombocytopenia in one case. Required tests for positive diagnosis and distinction between the primary categories of VWD are available. Specialized tests will allow a best characterization variants type 2 VWD for a better therapeutic approach.
authors.forename: Fatma
authors.initials: F
authors.lastname: Ben Lakhal
authors.forename: Wijdene
authors.initials: W
authors.lastname: El Borgi
authors.forename: Emna
authors.initials: E
authors.lastname: Gouider
authors.forename: Balkis
authors.initials: B
authors.lastname: Meddeb
authors.forename: Naouel
authors.initials: N
authors.lastname: Ben Salah
authors.forename: Raouf
authors.initials: R
authors.lastname: Hafsia