From the Guidelines
Quebec platelet disorder (QPD) is characterized by delayed-onset bleeding, typically occurring 12-24 hours after injury or trauma, with symptoms including easy bruising, nosebleeds, prolonged bleeding from cuts, heavy menstrual bleeding, and excessive bleeding after surgery or dental procedures. The main symptoms of QPD include:
- Easy bruising
- Nosebleeds
- Prolonged bleeding from cuts
- Heavy menstrual bleeding
- Excessive bleeding after surgery or dental procedures Patients may also experience joint bleeding, gastrointestinal bleeding, and hematuria (blood in urine) 1. Unlike other platelet disorders, QPD has a distinctive delayed bleeding pattern rather than immediate bleeding after injury. This delay occurs because QPD affects the storage of certain proteins in platelets, particularly urokinase-type plasminogen activator, which leads to increased fibrinolysis (breakdown of blood clots) after initial clot formation 1. The disorder is inherited in an autosomal dominant pattern, meaning only one copy of the altered gene is needed to cause symptoms. Laboratory findings typically show normal platelet counts but abnormal platelet function tests. Diagnosis can be challenging as routine coagulation tests may appear normal, and specialized testing is often required to confirm QPD. Recent studies have highlighted the importance of recognizing and diagnosing bleeding disorders of unknown cause, which may be related to QPD, and the need for further research on the optimal management strategies for these patients 1.
From the Research
Symptoms of Quebec Platelet Disorder
The symptoms of Quebec platelet disorder, also known as Quebec bleeding disorder, include:
- Delayed-onset bleeding following surgery, dental procedures, or trauma 2, 3, 4, 5
- Bleeding that leads to lifestyle changes 4
- Bruises that spread lower or as large or larger than an orange 4
- Joint bleeds 4
- Bleeding longer than 24 hours after dental extractions or deep cuts 4
- Easy bruising, petechiae, epistaxis, and mucocutaneous bleeding 6
Bleeding Risks and Management
Individuals with Quebec platelet disorder have a higher likelihood of experiencing bleeding complications, which can be modified by the use of fibrinolytic inhibitors 4. A comprehensive team approach, including consultation with a hematologist and administration of antifibrinolytic agents with platelet transfusions, can help manage bleeding risks in patients with QPD 5.
Clinical Features
The clinical features of QPD are characterized by increased expression and storage of urokinase plasminogen activator in platelets, leading to accelerated clot lysis and delayed-onset bleeding 2, 3. The disorder is associated with a unique gain-of-function defect in fibrinolysis, which is thought to be central to the pathogenesis of QPD bleeding 2.