Grey platelet syndrome: evidence for alpha-granule localization of the platelet plasminogen activator inhibitor-1 pool.
(1991) Pediatric Hematology & Oncology — Vol. 8, n° 2, p. 111-120 (1991)
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- Abstract
- The case of an 11-year-old boy with grey platelet syndrome is described. Platelets had the typical grey and ghostly appearance on May-Grünwald/Giemsa staining, caused by the absence of alpha granules confirmed by electron microscopy. Alpha granule protein content, i.e., beta-thromboglobulin and platelet factor 4, was less than 3% of normal and alpha granule secretion in response to thrombin was not detectable photometrically. The plasminogen activator inhibitor-1 pool in the patient's platelets was 5% of normal, confirming previous indirect evidence for the storage of this protein within the alpha-granule. Dense body secretion of adenosine triphosphate and 5-hydroxytryptamine was normal. Aggregation occurred normally in response to adenosine diphosphate and there was a slight delay in response to collagen.
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Vermylen, C., Vermylen, J., Hoet, B., Scheiff, J.-M., Ninane, J., & Cornu, G. (1991). Grey platelet syndrome: evidence for alpha-granule localization of the platelet plasminogen activator inhibitor-1 pool. Pediatric Hematology & Oncology, 8(2), 111-120. https://doi.org/10.3109/08880019109033438 (Original work published 1991)