Hemophilia A Clinical Trial
Official title:
GLOBAL HEMOSTATIC METHODS IN HEMOPHILIA AND VON WILLEBRAND'S DISEASE CORRELATION WITH PATIENTS' CLINICAL STATUS AND USEFULNESS FOR TREATMENT MONITORING
Patients with hemophilia who have the same level of deficient factor(s) may express
different severity of clinical presentation and bleeding tendency. Therefore a test which
could determine overall hemostasis rather than simple concentration of a single deficient
factor may correlate better with clinical phenotype in these patients.
The investigators will therefore study the usefulness of global hemostatic methods
(endogenous thrombin potential (ETP), overall hemostatic potential (OHP), fibrin clot
structure) and microparticles in the prediction of severity of bleeding and estimation of
response to the treatment in patients with hemophilia.
Since hemophilia patients on prophylactic treatment virtually do not bleed, additional
patients who are treated on demand only will be included enabling to study possible
modulatory effects of different hemostatic factors (particularly prothrombotic and thrombin
activatable fibrinolysis inhibitor (TAFI)) on clinical presentation. The investigators will
correlate both those factors and clinical severity with global hemostatic methods.
The investigators expect to prove that individual tailoring of the treatment, which may
enable lowering the prophylactic dose of factor concentrate without increasing the risk of
bleeding, is justified in some hemophilia patients. This approach would reduce the amount of
necessary factor concentrate in certain patients and decrease the cost (which represents
extensive burden for health care systems) of treatment without potential risk for the
patients.
n/a
Observational Model: Case Control, Time Perspective: Cross-Sectional
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