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Summary: Three patients with congenital macrothrombocytic thrombocytopenia are described. The platelets had a reduced electrophoretic mobility, and the content of sialic acid was also abnormally low, suggesting a defect in the platelet membrane. Platelets were rapidly removed from the circulation, possibly because the platelet membrane defect was associated with reduced electrostatic repulsive forces.
Grøttum et al. (Sat,) studied this question.