Usefulness of global tests of primary hemostasis in the initial screening of mild/moderate bleeding disorders for orienting towards von Willebrand disease or inherited platelet functions disorders

Minor bleeding manifestations are associated with several inherited hemorrhagic disorders collectively defined mild/moderate bleeding disorders (MMBD), including type 1 von Willebrand disease (VWD), platelet function disorders (PFD) and mild to moderate clotting factor deficiencies [ [1] Rodeghiero F. Pabinger I. Ragni M. Abdul-Kadir R. Berntorp E. Blanchette V. Bodó I. Casini A. Gresele P. Lassila R. Leebeek F. Lillicrap D. Mezzano D. Noris P. Srivastava A. Tosetto A. Windyga J. Zieger B. Makris M. Key N. Fundamentals for a systematic approach to mild and moderate inherited bleeding disorders: an EHA consensus report. Hemasphere. 2019; 3e286 Crossref PubMed Scopus (46) Google Scholar ]. The differentiation of these patients from hemostatically normal subjects is a difficult task and requires a careful clinical evaluation, including the application of standardized bleeding assessment tools (BAT) and the performance of some initial laboratory screening tests [ [2] Gresele P. Subcommittee on platelet physiology of the international society on thrombosis and hemostasis. Diagnosis of inherited platelet function disorders: guidance from the SSC of the ISTH. J. Thromb. Haemost. 2015; 13: 314-322 Abstract Full Text Full Text PDF PubMed Scopus (202) Google Scholar ]. Among the latter, methods measuring the overall haemostatic potential of whole blood, like the skin bleeding time (BT) and the PFA-100®, are still used for suspected mucocutaneous MMBD [ [3] Gresele P. Harrison P. Bury L. Falcinelli E. Gachet C. Hayward C.P. Kenny D. Mezzano D. Mumford A.D. Nugent D. Nurden A.T. Orsini S. Cattaneo M. Diagnosis of suspected inherited platelet function disorders: results of a worldwide survey. J. Thromb. Haemost. 2014; 12: 1562-1569 Abstract Full Text Full Text PDF PubMed Scopus (129) Google Scholar ], although their clinical usefulness remains controversial and thus are not currently recommended to orient further investigations [ 2 Gresele P. Subcommittee on platelet physiology of the international society on thrombosis and hemostasis. Diagnosis of inherited platelet function disorders: guidance from the SSC of the ISTH. J. Thromb. Haemost. 2015; 13: 314-322 Abstract Full Text Full Text PDF PubMed Scopus (202) Google Scholar , 3 Gresele P. Harrison P. Bury L. Falcinelli E. Gachet C. Hayward C.P. Kenny D. Mezzano D. Mumford A.D. Nugent D. Nurden A.T. Orsini S. Cattaneo M. Diagnosis of suspected inherited platelet function disorders: results of a worldwide survey. J. Thromb. Haemost. 2014; 12: 1562-1569 Abstract Full Text Full Text PDF PubMed Scopus (129) Google Scholar ].