Laboratory issues in bleeding disorders
Authors:
- 27 April 2006
- journal article
- review article
- Published by Wiley in Haemophilia
- Vol. 12 (s3) , 68-75
Abstract
Summary. 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.Keywords
This publication has 33 references indexed in Scilit:
- Linkage analysis in families diagnosed with type 1 von Willebrand disease in the European study, molecular and clinical markers for the diagnosis and management of type 1 VWDJournal of Thrombosis and Haemostasis, 2006
- Platelet function analyzer (PFA)‐100® closure time in the evaluation of platelet disorders and platelet functionJournal of Thrombosis and Haemostasis, 2006
- Epidemiology of von Willebrand Disease in Developing CountriesSeminars in Thrombosis and Hemostasis, 2005
- World Federation of Haemophilia Programs in Developing CountriesSeminars in Thrombosis and Hemostasis, 2005
- Provisional criteria for the diagnosis of VWD type 1: on behalf of the ISTH SSC Subcommittee on von Willebrand FactorJournal of Thrombosis and Haemostasis, 2005
- ELISA for factor VIII antibodies: does it detect antibodies much before the conventional Bethesda assay?Haemophilia, 2003
- Laboratory Diagnosis of von Willebrand DisorderAmerican Journal of Clinical Pathology, 2003
- Acquired von Willebrand syndromes: clinical features, aetiology, pathophysiology, classification and managementBest Practice & Research Clinical Haematology, 2001
- Factor IX Inhibitors and Anaphylaxis in Hemophilia BJournal of Pediatric Hematology/Oncology, 1997
- A Method for the Study of Antihaemophilic Globulin Inhibitors with Reference to Six CasesBritish Journal of Haematology, 1959