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Laboratory studies for paroxysmal nocturnal hemoglobinuria, with emphasis on flow cytometry

dc.contributor.authorLima, Margarida
dc.date.accessioned2022-07-11T14:45:35Z
dc.date.available2022-07-11T14:45:35Z
dc.date.issued2020-03-10
dc.description.abstractParoxysmal nocturnal hemoglobinuria (PNH) is a rare acquired clonal hematopoietic stem cell disorder caused by somatic mutations in the PIG-A gene, leading to the production of blood cells with absent or decreased expression of glycosylphosphatidylinositol-anchored proteins, including CD55 and CD59. Clinically, PNH is classified into three variants: classic (hemolytic), in the setting of another specified bone marrow disorder (such as aplastic anemia or myelodysplastic syndrome) and subclinical (asymptomatic). PNH testing is recommended for patients with intravascular hemolysis, acquired bone marrow failure syndromes and thrombosis with unusual features. Despite the availability of consensus guidelines for PNH diagnosis and monitoring, there are still discrepancies on how PNH tests are carried out, and these technical variations may lead to an incorrect diagnosis. Herein, we provide a brief historical overview of PNH, focusing on the laboratory tests available and on the current recommendations for PNH diagnosis and monitoring based in flow cytometry.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationLima M. Laboratory studies for paroxysmal nocturnal hemoglobinuria, with emphasis on flow cytometry. Pract Lab Med. 2020;20:e00158. doi:10.1016/j.plabm.2020.e00158pt_PT
dc.identifier.doi10.1016/j.plabm.2020.e00158pt_PT
dc.identifier.issn2352-5517
dc.identifier.urihttp://hdl.handle.net/10400.16/2697
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherElsevierpt_PT
dc.relation.publisherversionhttps://www.sciencedirect.com/science/article/pii/S2352551719300927?via%3Dihubpt_PT
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/pt_PT
dc.subjectBone marrow failure syndromespt_PT
dc.subjectFlow cytometrypt_PT
dc.subjectGlycosylphosphatidylinositolpt_PT
dc.subjectIntravascular hemolysispt_PT
dc.subjectPNHpt_PT
dc.subjectParoxysmal nocturnal hemoglobinuriapt_PT
dc.titleLaboratory studies for paroxysmal nocturnal hemoglobinuria, with emphasis on flow cytometrypt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.conferencePlaceNetherlandspt_PT
oaire.citation.startPagee00158pt_PT
oaire.citation.titlePractical Laboratory Medicinept_PT
oaire.citation.volume20pt_PT
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT

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