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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.
HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY Bleeding risks associated with inheritance of the Quebec platelet disorderFrom the Department of Pathology and Molecular Medicine and the Department of Medicine, McMaster University, Hamilton, ON, Canada; and the Department of Hematology and Oncology, Hôpital Sainte Justine, Montreal, QC, Canada.
Quebec platelet disorder (QPD) is an autosomal dominant bleeding disorder associated with increased urokinase-type plasminogen activator in platelets and
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| Copyright © 2004 by American Society of Hematology Online ISSN: 1528-0020 | |||||||||
-granule protein degradation. To determine bleeding risks and common manifestations of QPD, a history questionnaire was developed and administered to 127 relatives in a family with QPD. Data entry was done blinded to affected and unaffected status, determined by assays for platelet urokinase-type plasminogen activator (u-PA) and fibrinogen degradation. Odds ratios (ORs), with 95% confidence intervals (CIs), were determined for items queried. Summative bleeding scores for each individual were calculated using items with OR more than 1. Mean ages (34 years; range, 1-89 years) were similar for affected (n = 23) and unaffected (n = 104) family members. Affected individuals had higher mean bleeding scores (P < .0001) and a much higher likelihood (OR > 20) of having bleeding that led to lifestyle changes, bruises that spread lower or as large or larger than an orange or both, joint bleeds, bleeding longer than 24 hours after dental extractions or deep cuts, and received or been recommended other treatments (fibrinolytic inhibitors) for bleeding. Individuals with QPD and exposure(s) to hemostatic challenges had experienced excessive bleeding only when fibrinolytic inhibitors had not been used. These data illustrate that QPD is associated with increased risks of bleeding that can be modified by fibrinolytic inhibitors. 
