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Diagnose des Klons der paroxysmalen nächtlichen Hämoglobinurie

Diagnose des Klons der paroxysmalen nächtlichen Hämoglobinurie

Ikram Dammak / Nour Louati

61,63 €
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Editorial:
KS OmniScriptum Publishing
Año de edición:
2025
Materia
Medicina: cuestiones generales
ISBN:
9786208756994
61,63 €
IVA incluido
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Sobre IBD

Die Studie war retrospektiv und umfasste alle Anfragen zur PNH-Klonforschung, die im Durchflusszytometrielabor des CRTS von Sfax über einen Zeitraum von 2004 bis 2017 eingingen. Für jeden Patienten und/oder Zeugen des Tages wurde eine Probe peripheren venösen Blutes in Röhrchen mit EDTA entnommen und direkt an das Labor geschickt. Die Suche nach dem PNH-Klon durch CMF bestand aus der Analyse der Expression von GPI-verankerten Molekülen: CD16 und CD66b auf Granula und CD14 auf Monozyten. Das Vorhandensein eines HPN-Klons blieb erhalten, wenn mindestens zwei Zellpopulationen (Monozyten und PNNs) einen Mangel in der Expression von GPI-verankerten Molekülen aufwiesen. Die Defizitschwelle für GPI-verankerte Moleküle wurde auf 5 % festgelegt.In unserer Serie wurden 12 Fälle von PNH-Klonen diagnostiziert und der CD14-Expressionsmangel betrug 25 % (5,1-91 %). Die von CD66b und CD16 betrugen 44 % (5,2-100 %) bzw. 36 % (0-94 %).

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