[Monocentric study of Willebrand's disease in Tunisia: assets and difficulties]

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

[Immunophenotyping in adult acute myeloid leukemia: which prognostic value?]

Immunophenotyping is an essential step in the diagnosis of acute myeloid leukemia. Its prognostic value remains controversial with contradictory results. To assess prognostic impact of the immunophenotyping in AML. Our study is retrospective [October, 2005 - July, 2007] concerning 56 cases of AML [AML3 excluded] of the adult from 18 to 55 years old diagnosed and treated in Tunis Aziza Othmana Hospital. The immunophenotyping was performed by flow cytometry [Beckman Coulter EPICS XL MCL [registered sign]. We studied clinical and biological characteristic, immunophenotypic expressions, and parameters of the response to the treatment: complete remission [CR], overall survival [OS], relapse free survival [RFS] and relapse in a delay of 1 year and 2 years. SPSS software was used to perform the statistical analysis. The median age of the patients is of 37.7 +/- 11.8 years. Sex-ratio [M/F] is 1.33. Among individual antigenic expressions, only CD7 is associated to lower CR rates [p=0.044]. We did not find any statistically significant association between immunophenotypic expressions and OS nor with relapse or RFS. The impact of immunophenotyping in AML remains controversial because of contradictory results. The research of molecular changes would be an interesting alternative in our context