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MPS is characterized by autosomal dominant inheritance thrombocytopenia (5,000-120,000 μl-1), abnormally large platelets on peripheral blood smear, spontaneous aggregation (SA), normal clot retraction and thromboplastin formation, nypervolumetric shape change and normal amounts of plasma membrane. Here we describe further characteristics of MPS for 3 siblings /5 affected family members. The ultrastructural features of MPS platelets were normal except for the high frequency of giant granules. Platelet aggregation (PA) was studied in citrated platelet-rich plasma at platelet counts of 5,000-40,000 μl-1 and was quantitated microscopically from the decrease in single platelet count. PA could be increased over that due to SA alone by the addition of ADP, adrenalin, collagen, ionophore A23187, arachidonic acid and ristocetin, suggesting that the response to these agents may be normal. The ristocetin-induced increase in PA was completely blocked by an IgG specific for BSS. In contrast, over a 4 year period, thrombin (0.2 units/ml) either did not or only slightly increased PA over SA for 3/3 donors. However, MPS platelets pelleted from ACD-PRP and resuspended in Tyrodes’, pH 7.4 at a platelet count of ~ 200,000 μ1-1 showed a normal response to thrombin by aggregometry. Only one donor’s platelets had both reduced sialic acid and glycoprotein (G.P.) 1 reduction/abnormality with otherwise normal G.P. and protein profile, while the other 2 siblings’ platelets showed no such abnormalities. The above results indicate that biochemical variability exists for MPS platelets from different affected siblings. Moreover, our observations raise the possibility that platelet aggregation abnormalities in thrombobytopenic disorders (may be obscured by (1) preparation artefacts and/or (2) artificially increasing platelet counts for in vitro studies.

Montreal Platelet Syndrome (MPS):Ultrastructural, Biochemical And Functional Studies