Laboratory issues in bleeding disorders.
The clinical history of the patient and of his/her relatives is the most important tool for making correct diagnosis of inherited or acquired bleeding disorders. Several attempts have been made by clinicians to evaluate the sensitivity and specificity of bleeding symptoms. Specific and detailed questionnaires have been designed to quantify the bleeding tendency of patients with von Willebrand's disease (VWD) and a bleeding score has been calculated. VWD is considered the most frequent inherited bleeding disorder according to population studies: however, due to the complexity of its diagnosis, the number of patients with correct diagnosis of VWD in many developing countries is relatively low and most cases remain still under- or misdiagnosed. Once bleeding history is carefully evaluated by means of a bleeding score, the laboratory workout should be organized to find out the specific defect of haemostasis responsible for bleeding. Since factors involved in haemostasis are many, the correct approach must include first level screening tests with the aim to identify the abnormal phase of haemostasis involved: then, second level tests should be focused on the specific factors within the abnormal step of haemostasis. Among many other acquired bleeding disorders related to clinical conditions or to the use of drugs, the acquired inhibitors of haemostasis are rare but should be immediately characterized by appropriate laboratory tests because they can be often life-threatening for the patients.
Lillicrap
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D
Department of Pathology and Molecular Medicine, Queen's University, Kingston, Ontario, Canada.
Nair
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SC
Srivastava
A
A
Rodeghiero
F
F
Pabinger
I
I
Federici
A B
AB