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Grey platelet syndrome: evidence for alpha-granule localization of the platelet plasminogen activator inhibitor-1 pool.

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Vermylen, Christiane [UCL] Vermylen, J. Hoet, B. Scheiff, Jean-Marie [UCL] Ninane, J. Cornu, Guy [UCL]

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.

Document type Article de périodique (Journal article) – Case Reports, Journal Article, Research Support, Non-U.S. Gov't
Publication date 1991
Journal information "Pediatric hematology and oncology" - Vol. 8, no. 2, p. 111-20 (1991)
Peer reviewed yes
issn 0888-0018
Publication status Publié
Affiliation UCL - MD/MNOP - Département de morphologie normale et pathologique
MESH Subject Child ; Cytoplasmic Granules - chemistry ; Eosine Yellowish-(YS) ; Humans ; Male ; Methylene Blue ; Plasminogen Inactivators - blood ; Platelet Aggregation ; Platelet Factor 4 - deficiency ; Staining and Labeling ; Syndrome ; Thrombocytopenia - metabolism ; beta-Thromboglobulin - deficiency
Links
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Bibliographic reference Vermylen, Christiane ; Vermylen, J. ; Hoet, B. ; Scheiff, Jean-Marie ; Ninane, J. ; et. al. Grey platelet syndrome: evidence for alpha-granule localization of the platelet plasminogen activator inhibitor-1 pool.. In: Pediatric hematology and oncology, Vol. 8, no. 2, p. 111-20 (1991)
Permanent URL http://hdl.handle.net/2078.1/27210