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...

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Bibliographic Details
Published inPlatelets (Edinburgh) Vol. 17; no. 1; pp. 14 - 19
Main Authors Chedani, Hicham, Dupuy, Evelyne, Massé, Jean-Marc, Cramer, Elisabeth M.
Format Journal Article
LanguageEnglish
Published England Informa UK Ltd 01.02.2006
Taylor & Francis
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
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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.
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ISSN:0953-7104
1369-1635
DOI:10.1080/09537100500165601