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
in Chinese
| 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>
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Pathology
/
Thrombocytopenia
/
Blood Platelets
/
Microscopy, Immunoelectron
/
Platelet Glycoprotein GPIb-IX Complex
/
Platelet Membrane Glycoprotein IIb
/
Integrin beta3
/
Genetics
/
Metabolism
Limits:
Adult
/
Female
/
Humans
Language:
Chinese
Journal:
Chinese Journal of Hematology
Year:
2006
Type:
Article
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