Multidisciplinary clinical management of paroxysmal nocturnal hemoglobinuria

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dc.contributor.authorSahin, Fahri
dc.contributor.authorOzkan, Melda Comert
dc.contributor.authorMete, Nihal Gokmen
dc.contributor.authorYilmaz, Mumtaz
dc.contributor.authorOruc, Nevin
dc.contributor.authorGurgun, Alev
dc.contributor.authorKayikcioglu, Meral
dc.contributor.authorGuler, Ayse
dc.contributor.authorGokcay, Figen
dc.contributor.authorBilgir, Ferda
dc.contributor.authorCeylan, Cengiz
dc.contributor.authorBilgir, Oktay
dc.contributor.authorSari, Ismail Hakan
dc.contributor.authorSaydam, Güray
dc.date.accessioned2019-10-27T22:29:24Z
dc.date.available2019-10-27T22:29:24Z
dc.date.issued2015
dc.departmentEge Üniversitesien_US
dc.description.abstractParoxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired disease caused by clonal expansion of one or more hematopoietic stem cell (HSC) lines due to a somatic mutation of the phosphatidylinositol glycan anchor (PIG-A) gene located on Xp22.1. PNH incidence is 1.5-2 cases per million of the population per year. PNH can affect multiple systems in the body and requires multidisciplinary clinical management. Patients can manifest with severe pancytopenia, life-threatening thrombosis affecting the hepatic, abdominal, cerebral, and subdermal veins, and high requirements for blood transfusion due to haemolytic anemia. PNH can also be associated with bone marrow failure. Advances in diagnostic techniques and a targeted therapeutic approach for PNH have emerged in the last two decades. Eculizumab, a promising humanized monoclonal antibody against C5, is the first approved therapy for PNH.en_US
dc.identifier.endpage9en_US
dc.identifier.issn2160-1992
dc.identifier.issue1en_US
dc.identifier.pmid26171279en_US
dc.identifier.startpage1en_US
dc.identifier.urihttps://hdl.handle.net/11454/51095
dc.identifier.volume5en_US
dc.identifier.wosWOS:000361130500001en_US
dc.identifier.wosqualityN/Aen_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherE-Century Publishing Corpen_US
dc.relation.ispartofAmerican Journal of Blood Researchen_US
dc.relation.publicationcategoryMakale - Ulusal Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectParoxysmal nocturnal hemoglobinuriaen_US
dc.subjectdiagnosisen_US
dc.subjecttreatmenten_US
dc.subjecteculizumaben_US
dc.titleMultidisciplinary clinical management of paroxysmal nocturnal hemoglobinuriaen_US
dc.typeReview Articleen_US

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