The Montreal Platelet Syndrome (MPS) is a hereditary platelet disorder with a mild bleeding diathesis.


Genetic defect: defect in platelet calpain, a calcium-activated neutral protease


Inheritance: autosomal dominant


Clinical features:

(1) mild bleeding disorder

(2) family history


Laboratory features:

(1) large and giant platelets (macrothrombocytes)

(2) moderate thrombocytopenia

(3) prolonged bleeding time

(4) normal clot retraction

(5) spontaneous aggregation of platelets in platelet-rich plasma at pH 7.4


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