Thromb Haemost 1986; 56(01): 066-070
DOI: 10.1055/s-0038-1661605
Original Article
Schattauer GmbH Stuttgart

Megakaryocytes from the Marrow of a Patient with Glanzmann’s Thrombasthenia Lacked GPII b-III a Complexes

P Hourdillé
The Laboratoire d’Hémobiologie, Hôpital Cardiologique Pessac Bordeaux, Paris, France
,
P Fialon
The Laboratoire d’Hémobiologie, Hôpital Cardiologique Pessac Bordeaux, Paris, France
,
F Belloc
The Laboratoire d’Hémobiologie, Hôpital Cardiologique Pessac Bordeaux, Paris, France
,
M Namur
The Laboratoire d’Hémobiologie, Hôpital Cardiologique Pessac Bordeaux, Paris, France
,
M R Boisseau
The Laboratoire d’Hémobiologie, Hôpital Cardiologique Pessac Bordeaux, Paris, France
,
A T Nurden
*   The INSERM U 150, Hôpital Lariboisière, Paris, France
› Author Affiliations
Further Information

Publication History

Received 08 April 1986

Accepted after revision 26 May 1986

Publication Date:
13 July 2018 (online)

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Summary

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 adsorbed 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.