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Artículo

Megakaryocytic emperipolesis and platelet function abnormalities in five patients with gray platelet syndrome

Larroca, Luigi M.; Heller, Paula GracielaIcon ; Podda, Gianmarco; Pujol Moix, Nuria; Glembotsky, Ana ClaudiaIcon ; Pecci, Alessandro; Alberelli, Maria Adele; Balduini, Carlo L.; Landolfi, Raffaele; Cattaneo, Marco; De Candia, Erica
Fecha de publicación: 2015
Editorial: Taylor & Francis Ltd
Revista: Platelets
ISSN: 0953-7104
e-ISSN: 1369-1635
Idioma: Inglés
Tipo de recurso: Artículo publicado
Clasificación temática:
Salud Ocupacional

Resumen

The gray platelet syndrome (GPS) is a rare congenital platelet disorder characterized by mild to moderate bleeding diathesis, macrothrombocytopenia and lack of azurophilic α-granules in platelets. Some platelet and megakaryocyte (MK) abnormalities have been described, but confirmative studies of the defects in larger patient cohorts have not been undertaken. We studied platelet function and bone marrow (BM) features in five GPS patients with NBEAL2 autosomal recessive mutations from four unrelated families. In 3/3 patients, we observed a defect in platelet responses to protease-activated receptor (PAR)1-activating peptide as the most consistent finding, either isolated or combined to defective responses to other agonists. A reduction of PAR1 receptors with normal expression of major glycoproteins on the platelet surface was also found. Thrombin-induced fibrinogen binding to platelets was severely impaired in 2/2 patients. In 4/4 patients, the BM biopsy showed fibrosis (grade 2-3) and extensive emperipolesis, with many (36-65%) MKs containing 2-4 leukocytes engulfed within the cytoplasm. Reduced immunolabeling for platelet factor 4 together with normal immunolabeling for CD63 in MKs of two patients demonstrated that GPS MKs display an alpha granule-specific defect. Increased immunolabeling for P-selectin and decreased immunolabeling for PAR1, PAR4 and c-MPL were also observed in MKs of two patients. Marked emperipolesis, specific defect of MK alpha-granule content and defect of PAR1-mediated platelet responses are present in all GPS patients that we could study in detail. These results help to further characterize the disease.
Palabras clave: Emperipolesis , Par1 Receptor , Gray Platelet Syndrome , Megakaryocytes
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info:eu-repo/semantics/openAccess Excepto donde se diga explícitamente, este item se publica bajo la siguiente descripción: Creative Commons Attribution-NonCommercial-ShareAlike 2.5 Unported (CC BY-NC-SA 2.5)
Identificadores
URI: http://hdl.handle.net/11336/38762
DOI: http://dx.doi.org/10.3109/09537104.2014.994093
URL: https://www.tandfonline.com/doi/abs/10.3109/09537104.2014.994093
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Citación
Larroca, Luigi M.; Heller, Paula Graciela; Podda, Gianmarco; Pujol Moix, Nuria; Glembotsky, Ana Claudia; et al.; Megakaryocytic emperipolesis and platelet function abnormalities in five patients with gray platelet syndrome; Taylor & Francis Ltd; Platelets; 26; 8; 2015; 751-757
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