Monocentric study of Willebrand’s disease in tunisia: assets and difficulties

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Published Dec 29, 2015
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Vol. 93 No. 10 (2015): Issue Oct 2015

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Fatma Ben Lakhal
Wijdene El Borgi
Emna Gouider
Balkis Meddeb
Naouel Ben Salah
Raouf Hafsia

Abstract

SUMMARY
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.
Aim : 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 FVIII: 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.

Keywords:

Von Willebrand disease, Von Willebrand factor, Biological diagnosis, Difficulties.

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