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Blood, 1 July 2004, Vol. 104, No. 1, pp. 159-165.
Prepublished online as a Blood First Edition Paper on March 16, 2004; DOI 10.1182/blood-2003-11-4077.
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Submitted December 1, 2003
Accepted March 5, 2004
Bleeding Risks Associated with Inheritance of the Quebec Platelet Disorder
Heather McKay, Francine Derome, M A Haq, Susan Whittaker, Emmy Arnold, Frederic Adam, Nancy M Heddle, Georges E Rivard, and Catherine P Hayward*
Pathology and Molecular Medicine, McMaster University, Hamilton, Ontario, Canada
Hematology/Oncology, Hopital Sainte Justine, Montreal, Quebec, Canada
Medicine, McMaster University, Hamilton, Ontario, Canada
Pathology and Molecular Medicine, McMaster University, Hamilton, Ontario, Canada; Medicine, McMaster University, Hamilton, Ontario, Canada
* Corresponding author; email: haywrdc{at}mcmaster.ca.
The Quebec Platelet Disorder (QPD) is an autosomal dominant bleeding disorder associated with increased urokinase-type plasminogen activator in platelets and  -granule protein degradation. To determine bleeding risks and common manifestations of the QPD, a history questionnaire was developed and administered to 127 relatives in a family with the QPD. Data entry was done blinded to affected/unaffected status, determined by assays for platelet u-PA and fibrinogen degradation. Odds ratios (OR), with 95% confidence intervals, were determined for items queried. Summative bleeding scores for each individual were calculated using items with OR>1. Mean ages (34 years; range 1-89) were similar for affected (n=23) and unaffected (n=104) family members. Affected individuals had higher mean bleeding scores (p<0.0001) and a much higher likelihood (OR>20) of having: bleeding leading to lifestyle changes, bruises that spread lower and/or as large/larger than an orange; joint bleeds; bleeding > 24 hours after dental extractions or deep cuts; and received/been recommended other treatments (fibrinolytic inhibitors) for bleeding. Individuals with the QPD and exposure(s) to hemostatic challenges had experienced excessive bleeding only when fibrinolytic inhibitors had not been used. These data illustrate that the QPD is associated with increased risks for bleeding that can be modified by fibrinolytic inhibitors.
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