Quebec Platelet Disorder (QPD) is a rare bleeding disorder associated with a hereditary platelet defect.


Synonym: Factor V Quebec, BDPLT5


Chromosome location: 10q22.2

Gene affected: PLAU (plasminogen activator, urokinase), a serine protease

Mechanism: gain-of-function defect in fibrinolysis, with a degradation of alpha-granule proteins and accelerated clot lysis


Inheritance: autosomal dominant


Clinical features:

(1) familial bleeding disorder

(2) delayed bleeding (12 to 4 days after a hemostatic challenge), often prolonged

(3) joint bleeds with or without arthropathy

(4) other signs of bleeding disorder: bruising, nosebleeds, hematuria, abnormal bleeding after dental extraction, excessive bleeding after trauma, menorrhagia

(5) problems with wound healing

(6) variable need for transfusion because of bleeding

(7) positive response to a fibrinolytic inhibitor (tranexamic acid or aminocaproic acid)


Laboratory findings:

(1) variable thrombocytopenia

(2) variable iron deficiency from recurrent bleeding

(3) tests performed on plasma are usually normal



(1) venous thrombotic event during therapy with fibrinolytic inhibitor


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