[Report of a patient with spontaneous aggregation of his giant and morphologically abnormal platelets].
OBJECTIVE
To study the pathological and clinical characteristics of a patient with spontaneous platelet aggregation of his giant and morphologically abnormal platelets.


METHODS
Platelet size and structure were observed under light microscope and electron microscope. Platelet aggregation was measured turbidometrically. Platelet glycoproteins (GP) were analyzed using flow cytometry. PCR and DNA sequencing were performed to identify the gene abnormality.


RESULTS
The patient had spontaneous platelet aggregation of giant platelets with thickened plasma membrane and increased number of granules in various shapes. Aspirin and ticlopidine did not affect the spontaneous aggregation. The expression of GP I b, GP II b, GP III a and P-selectin in the platelet membrane were in normal range. Results of gene analyses for GP I balpha, GP I bbeta and GPIX were also normal.


CONCLUSIONS
Both morphological and functional abnormalities of the platelets from the patient were clearly distinguishable from that of other hereditary giant platelet disorders. It would probably represent a novel platelet disorder which had not been reported to date.
Wang
Zhaoyue
Z
Jiangsu Institute of Hematology, The First Affiliated Hospital of Suzhou University, Suzhou 215006, China.
Shi
Jumei
J
Han
Yue
Y
Wang
Yingchun
Y
Bai
Xia
X
Lu
Dingwei
D
Ruan
Changgeng
C
D001241:Aspirin / Q000494:pharmacology; D001606:Bernard-Soulier Syndrome / Q000378:metabolism / Q000473:pathology*; D001791:Blood Platelet Disorders / Q000378:metabolism / Q000473:pathology*; D048429:Cell Size / Q000502:physiology; D002648:Child; D003594:Cytoplasmic Granules / Q000473:pathology / Q000648:ultrastructure; D005260:Female; D006801:Humans; D010974:Platelet Aggregation / Q000187:drug effects / Q000502:physiology*; D010975:Platelet Aggregation Inhibitors / Q000494:pharmacology; D010980:Platelet Membrane Glycoproteins / Q000235:genetics / Q000378:metabolism*; D013988:Ticlopidine / Q000494:pharmacology
D010975:Platelet Aggregation Inhibitors; D010980:Platelet Membrane Glycoproteins; D013988:Ticlopidine; D001241:Aspirin