A preliminary study of an inherited macrothrombocytopenia disorder with abnormal large granules / 中华血液学杂志
Chinese Journal of Hematology
;
(12): 584-587, 2006.
Article
Dans Chinois
| WPRIM
| ID: wpr-328417
ABSTRACT
<p><b>OBJECTIVE</b>To study the platelet morphology and function of an inherited macrothrombocytopenia disorder with abnormal large granules.</p><p><b>METHODS</b>Platelet size and structure were investigated by both light microscopy and electron microscopy. The platelet membrane expression of GP I b, GP II b, GPIII a, P-selectin and CD63 were analyzed by using respective monoclonal antibodies. Platelet 5-hydroxy-tryptamine was measured with spectrophotofluorometer.</p><p><b>RESULTS</b>Both the patient and her father had large granules in their platelets, with exocytosis being easily observed. The expressions of GP I b, GP II b and GP II a on the platelets were in normal range, while P-selectin and CD63 were somewhat increased. The abnormal large granules were not the alpha granules, lysosomes or dense bodies.</p><p><b>CONCLUSION</b>Both morphological and functional abnormalities of the platelets from the patient are clearly distinguishable from other hereditary giant platelet disorders. It would probably represent a novel platelet disorder.</p>
Texte intégral:
Disponible
Indice:
WPRIM (Pacifique occidental)
Sujet Principal:
Anatomopathologie
/
Thrombopénie
/
Plaquettes
/
Microscopie immunoélectronique
/
Complexe glycoprotéique GPIb-IX plaquettaire
/
Glycoprotéine-IIb de membrane plaquettaire
/
Intégrine bêta3
/
Génétique
/
Métabolisme
Limites du sujet:
Adulte
/
Femelle
/
Humains
langue:
Chinois
Texte intégral:
Chinese Journal of Hematology
Année:
2006
Type:
Article
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