Neutrophil secretory defect in the gray platelet syndrome: A new case

We report the case of a 60-year-old woman who was newly diagnosed for the gray platelet syndrome (GPS). This patient had long-term thrombocytopenia which had been initially misdiagnosed as idiopathic thrombocytopenic purpura (ITP). Blood smear displayed characteristic gray platelets, allowing the di...

Full description

Saved in:
Bibliographic Details
Published in:Platelets (Edinburgh) 2006-02, Vol.17 (1), p.14-19
Main Authors: Chedani, Hicham, Dupuy, Evelyne, Massé, Jean-Marc, Cramer, Elisabeth M.
Format: Article
Language:English
Subjects:
Blood Platelet Disorders - blood
Blood Platelet Disorders - diagnosis
Blood Platelet Disorders - pathology
Blood Platelets - pathology
Blood Platelets - ultrastructure
Female
granulocytes
Gray platelet syndrome
Humans
Middle Aged
Neutrophils - metabolism
Neutrophils - pathology
Neutrophils - secretion
PMN
specific granules
Syndrome
ultrastructure
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:We report the case of a 60-year-old woman who was newly diagnosed for the gray platelet syndrome (GPS). This patient had long-term thrombocytopenia which had been initially misdiagnosed as idiopathic thrombocytopenic purpura (ITP). Blood smear displayed characteristic gray platelets, allowing the diagnosis to be made, which was confirmed by electron microscopy (EM). Polymorphonuclear neutrophils (PMN) appeared poorly granulated on the May-Grunwald-Giemsa-stained blood smear. Flow cytometry analysis of PMN demonstrated increased expression of CD35, CD11b and CD18 at resting PMN surface, without any changes after fMLP stimulation. Ultrastructural study retrieved a decreased number of myeloperoxidase (MPO)-negative secondary granules in PMN. Immunolabeling confirmed the presence of membrane proteins and the absence of soluble content in platelet and megakaryocyte (MK) -granules, and the decrease of secondary granules and secretory vesicles in PMN. This new observation demonstrates that the impairment of the secretory compartment of PMN is definitely a hallmark of GPS, and that the detection of these subtle abnormalities should be searched with adequate and up-to-date technical approaches.
ISSN:0953-7104
1369-1635
DOI:10.1080/09537100500165601