Article
Patients with paroxysmal nocturnal hemoglobinuria have a high frequency of peripheral-blood T cells expressing activating isoforms of inhibiting superfamily receptors.
Blood - 1 Oct 2005
Poggi Alessandro, Negrini Simone, Zocchi Maria Raffaella, Massaro Anna-Maria, Garbarino Lucia, Lastraioli Sonia, Gargiulo Lucia, Luzzatto Lucio, Notaro Rosario
Abstract excerpt
Patients with paroxysmal nocturnal hemoglobinuria (PNH) have a large clonal population of blood cells deriving from hematopoietic stem cells (HSCs) deficient in glycosylphosphatidylinositol (GPI)-anchored surface molecules. A current model postulates that PNH arises through negative selection against normal HSCs exerted by autoreactive T cells, whereas PNH HSCs escape damage. We have investigated the inhibitory...
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