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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Specialty: Genetics, Hematology Oncology
ICD-10: ,
