Megakaryocytes from the marrow of a patient with Glanzmann's thrombasthenia lacked GP IIb-IIIa complexes.
Thromb Haemost
; 56(1): 66-70, 1986 Aug 20.
Article
em En
| MEDLINE
| ID: mdl-3535160
ABSTRACT
Although it is recognized that glycoprotein (GP) IIb-IIIa complexes are deficient in platelets in Glanzmann's thrombasthenia, little is known of the origin of the defect. We have examined the megakaryocytes in a bone marrow aspirate obtained from a thrombasthenia patient during surgery. Analysis of platelet proteins by SDS-polyacrylamide gel electrophoresis confirmed the patient to be of the type I subgroup. The megakaryocytes were examined by immunofluorescence or by immunocytochemical procedures combined with electron microscopy. Antibodies used included the murine monoclonal antibody, AP-2 and the human allo-antibody, IgG L, both of which recognize determinants on GP IIb-IIIa complexes. Bound antibody was detected by anti-IgG antibodies coupled to fluorescein isothiocyanate or absorbed on gold particles. In the immunofluorescence studies, permeabilized megakaryocytes were identified by double staining using an antibody to von Willebrand factor (vWF). Whereas mature megakaryocytes and their small precursor cells from normal individuals were strongly fluorescent with AP-2 and IgG L, most vWF positive cells from the Glanzmann's thrombasthenia patient were negative and the remainder gave but a weak background fluorescence. Immunogold staining on the surface of marrow cells was severely reduced. Our results confirm a deficiency of GP IIb-IIIa complexes in megakaryocytes in thrombasthenia.
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Base de dados:
MEDLINE
Assunto principal:
Transtornos Plaquetários
/
Medula Óssea
/
Trombastenia
/
Megacariócitos
/
Glicoproteínas da Membrana de Plaquetas
Tipo de estudo:
Prognostic_studies
Limite:
Humans
Idioma:
En
Ano de publicação:
1986
Tipo de documento:
Article