[Flow cytometric analysis of platelets in patients with Glanzmann's thrombasthenia]. 1991

S Kinoshita, and K Yoshioka, and T Shindo, and M Kasahara, and K Tanaka
Department of Pediatrics, Osaka National Hospital.

Platelets from 10 patients with Glanzmann's thrombasthenia (7 patients with type I and 3 with type II) and their 18 family members (11 parents, 6 siblings and one daughter) were analyzed by flow cytometry using 3 different commercially available FITC-labeled monoclonal antibodies. The amount of platelet GPIIbIIIa was calculated by using the ratio of the fluorescence intensity of the mean channel in comparison to normal platelets. The amount of platelet GPIIbIIIa was lower than 19% in 6 patients with type I and one patient with type II thrombasthenia. One type I patient had a 46.5% GPIIbIIIa amount as assessed using the monoclonal antibody TP80 (Nichirei Corp. Japan) which recognized GPIIb, although the other 2 antibodies showed an amount of less than 5%. One type II patient showed the following results: 30.9% (TP80), 28.2% (P2 antibody, Immunotech, France), and 3.9% (PLT1, Coulter Immunology, USA). The remaining type II patient consistently showed a normal amount of platelet GPIIbIIIa using all antibodies, appeared to be a variant form of thrombasthenia. The parents of type I patients had a significantly lower amount of platelet GPIIbIIIa compared to normal and 2 siblings of type I patients were diagnosed as heterozygotes. These findings suggest that Glanzmann's thrombasthenia is more heterogeneous than we have previously suspected, and that flow cytometric analysis using different monoclonal antibodies is a useful tool for identifying those heterogeneities and for the detection of type I carriers.

UI MeSH Term Description Entries
D008297 Male Males
D010980 Platelet Membrane Glycoproteins Surface glycoproteins on platelets which have a key role in hemostasis and thrombosis such as platelet adhesion and aggregation. Many of these are receptors. PM-GP,Platelet Glycoprotein,Platelet Membrane Glycoprotein,PM-GPs,Platelet Glycoproteins,Glycoprotein, Platelet,Glycoprotein, Platelet Membrane,Glycoproteins, Platelet,Glycoproteins, Platelet Membrane,Membrane Glycoprotein, Platelet,Membrane Glycoproteins, Platelet,PM GP
D001792 Blood Platelets Non-nucleated disk-shaped cells formed in the megakaryocyte and found in the blood of all mammals. They are mainly involved in blood coagulation. Platelets,Thrombocytes,Blood Platelet,Platelet,Platelet, Blood,Platelets, Blood,Thrombocyte
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D005192 Family Health The health status of the family as a unit including the impact of the health of one member of the family on the family as a unit and on individual family members; also, the impact of family organization or disorganization on the health status of its members. Health, Family
D005260 Female Females
D005434 Flow Cytometry Technique using an instrument system for making, processing, and displaying one or more measurements on individual cells obtained from a cell suspension. Cells are usually stained with one or more fluorescent dyes specific to cell components of interest, e.g., DNA, and fluorescence of each cell is measured as it rapidly transverses the excitation beam (laser or mercury arc lamp). Fluorescence provides a quantitative measure of various biochemical and biophysical properties of the cell, as well as a basis for cell sorting. Other measurable optical parameters include light absorption and light scattering, the latter being applicable to the measurement of cell size, shape, density, granularity, and stain uptake. Cytofluorometry, Flow,Cytometry, Flow,Flow Microfluorimetry,Fluorescence-Activated Cell Sorting,Microfluorometry, Flow,Cell Sorting, Fluorescence-Activated,Cell Sortings, Fluorescence-Activated,Cytofluorometries, Flow,Cytometries, Flow,Flow Cytofluorometries,Flow Cytofluorometry,Flow Cytometries,Flow Microfluorometries,Flow Microfluorometry,Fluorescence Activated Cell Sorting,Fluorescence-Activated Cell Sortings,Microfluorimetry, Flow,Microfluorometries, Flow,Sorting, Fluorescence-Activated Cell,Sortings, Fluorescence-Activated Cell
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

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