Gray platelet syndrome : dissociation between abnormal sorting in megakaryocyte α-granules and normal sorting in Weibel-Palade bodies of endothelial cells

The gray platelet syndrome (GPS) is a rare congenital bleeding disorder in which megakaryocytes and platelets are deficient in alpha-granule secretory proteins. Since the Weibel-Palade bodies (WPB) of endothelial cells as well as the alpha-granules contain the von Willebrand Factor (vWF) and P-selec...

Full description

Saved in:
Bibliographic Details
Published in:The Journal of clinical investigation 1993-12, Vol.92 (6), p.3023-3028
Main Authors: GEBRANE-YOUNES, J, CRAMER, E. M, ORCEL, L, CAEN, J. P
Format: Article
Language:English
Subjects:
Biological and medical sciences
Biopsy
Blood Platelet Disorders - blood
Blood Platelet Disorders - pathology
Cytoplasmic Granules - pathology
Cytoplasmic Granules - ultrastructure
Endothelium, Vascular - pathology
Endothelium, Vascular - ultrastructure
Female
Hematologic and hematopoietic diseases
Humans
Male
Medical sciences
Megakaryocytes - pathology
Megakaryocytes - ultrastructure
Microscopy, Electron
Microscopy, Immunoelectron
P-Selectin
Platelet diseases and coagulopathies
Platelet Membrane Glycoproteins - analysis
Skin - blood supply
Skin - pathology
Syndrome
von Willebrand Factor - analysis
Citations: Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:The gray platelet syndrome (GPS) is a rare congenital bleeding disorder in which megakaryocytes and platelets are deficient in alpha-granule secretory proteins. Since the Weibel-Palade bodies (WPB) of endothelial cells as well as the alpha-granules contain the von Willebrand Factor (vWF) and P-selectin, we examined by transmission electron microscopy the dermis capillary network of two patients with GPS. Endothelial cells showed the presence of normal WPB with typical internal tubules. Using single and double immunogold labeling for vWF and P-selectin, we detected vWF within WPB, where it was codistributed with the tubules, whereas P-selectin delineated the outline of WPB. Therefore, the fundamental targeting defect in GPS is specific to the megakaryocytic cell line.
ISSN:0021-9738
1558-8238
DOI:10.1172/JCI116926