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Glanzmann thrombastenia

Antonija Vuk, Vladimir Stancić, Goran Rincić, Mario Ledinsky, Ljiljana Grbac, Nevenka Stancić
A 64-year-old female receiving clopidogrel and aspirin antiaggregation therapy after percutaneous coronary intervention for non-STEMI myocardial infarction developed nontraumatic bilateral subdural hematoma with dizziness, vertigo and headache. Craniotomy had to be postponed because of reduced ADP platelet aggregability. Four days after clopidogrel withdrawal and transfusion of 12 platelet concentrate units, ADP aggregation transiently normalized and bilateral trepanation with hematoma evacuation was performed...
June 2010: Acta Clinica Croatica
Filomena A Carvalho, Simon Connell, Gabriel Miltenberger-Miltenyi, Sónia Vale Pereira, Alice Tavares, Robert A S Ariëns, Nuno C Santos
The established hypothesis for the increase on erythrocyte aggregation associated with a higher incidence of cardiovascular pathologies is based on an increase on plasma adhesion proteins concentration, particularly fibrinogen. Fibrinogen-induced erythrocyte aggregation has been considered to be caused by its nonspecific binding to erythrocyte membranes. In contrast, platelets are known to have a fibrinogen integrin receptor expressed on the membrane surface (the membrane glycoprotein complex alpha(IIb)beta(3))...
August 24, 2010: ACS Nano
Sitki Goksu, Rauf Gul, Onder Ozen, Mehmet Yilmaz, Orhan Buyukbebeci, Unsal Oner
Glanzmann thrombastenia (GT) is a rare condition of an inherited autosomal recessive gene characterized with bleeding tendency. The condition is rarely met in the OR. and therefore it is essential that anesthesiologist be cognizant of the risk involved and be prepared with all necessary precautionary measures. We present a GT case in a 27-year-old male with a mass in the anticubital region of right wrist that was successfully excised using the non-invasive intravenous regional analgesia (IVRA). The use of platelet transfusion and the recombinant factor VIIa, are stressed...
February 2010: Middle East Journal of Anesthesiology
Kathleen Freson, Veerle Labarque, Chantal Thys, Christine Wittevrongel, Chris Van Geet
This review on platelet research focuses on defects of adhesion, cytoskeletal organisation, signal transduction and secretion. Platelet defects can be studied by different laboratory platelet functional assays and morphological studies. Easy bruising or a suspected platelet-based bleeding disorder is of course the most obvious reason to test the platelet function in a patient. However, nowadays platelet research also contributes to our understanding of human pathology in other disciplines such as neurology, nephrology, endocrinology and metabolic diseases...
December 2007: European Journal of Pediatrics
A Stepanian, P Nurden, P Bonnet, O Delhaume, J F Schved, C Biron
No abstract text is available yet for this article.
September 2001: Annales de Biologie Clinique
C M Hackeng, M W Pladet, J W Akkerman, H J van Rijn
Low density lipoprotein (LDL) is known to sensitize platelets to agonists via integrin mediated outside-in signaling (Hackeng, C. M., Huigsloot, M., Pladet, M. W., Nieuwenhuis, H. K., Rijn, H. J. M. v., and Akkerman, J. W. N. (1999) Arterioscler. Thromb. Vasc. Biol., in press). As outside in signaling is associated with phosphorylation of p125(FAK), the effect of LDL on p125(FAK) phosphorylation in platelets was investigated. LDL induced p125(FAK) phosphorylation in a dose- and time- dependent manner. The phosphorylation was independent of ligand binding to integrin alphaIIbbeta3 and aggregation, such in contrast to alpha-thrombin-induced p125(FAK) phosphorylation, that critically depended on platelet aggregation...
January 1, 1999: Journal of Biological Chemistry
R Dardik, Y Kaufmann, N Savion, N Rosenberg, B Shenkman, D Varon
The aim of our study was to explore the role of platelets and their specific integrin receptors in mediating the interaction of 4 human tumor cell lines (3 melanoma and 1 carcinoma) with the extracellular matrix (ECM) under static and arterial flow conditions. Under static conditions, all 4 cell lines adhered to the ECM. The adhesion capacity of all 4 cell lines was virtually abolished by application of flow during incubation with the ECM. Under static conditions, tumor cell adhesion was not affected by adding platelets to the cell suspension and was slightly reduced by pre-coating the ECM with platelets prior to the addition of tumor cells...
January 17, 1997: International Journal of Cancer. Journal International du Cancer
M Torti, G Ramaschi, F Sinigaglia, E G Lapetina, C Balduini
The stimulation of human platelets with physiological agonists results in the incorporation of several proteins into the cytoskeleton, fibrinogen binding, and platelet aggregation. We recently demonstrated that the Ras-related low molecular weight GTP-binding protein Rap2B associates with the cytoskeleton in activated platelets and that this interaction requires platelet aggregation. In the present study we demonstrate that agonist-induced actin polymerization is necessary for the translocation of Rap2B to the cytoskeleton, suggesting that Rap2B interacts with the newly formed actin filaments...
May 10, 1994: Proceedings of the National Academy of Sciences of the United States of America
G I Johnston, S Heptinstall
Extracellular Ca2+ ions are required for platelet aggregation and we show that they enter two platelet pools. One pool is rapidly filled and easily displaced by EGTA. The second is filled more slowly and is not displaced by EGTA. The EGTA-displaceable pool is believed to be surface-located and was found to contain at least one class of saturable binding sites as well as a class of non-saturable binding sites. The saturable sites were found to be highly selective for Ca2+ (dissociation constant, 3.5 X 10(-7) M) even in the presence of 1 mM Mg2+ ions, and they took up between 261,000 and 307,000 Ca2+ ions/platelet...
February 25, 1988: Thrombosis and Haemostasis
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