Necrotizing Fasciitis in Paroxysmal Nocturnal Hemoglobinuria

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dc.contributor.authorPatir, Pusem
dc.contributor.authorIsik, Yakup
dc.contributor.authorTurk, Yigit
dc.contributor.authorUgur, Mehmet Can
dc.contributor.authorCeylan, Cengiz
dc.contributor.authorGorgun, Gulnur
dc.contributor.authorGokmen, Nihal Mete
dc.contributor.authorSaydam, Güray
dc.contributor.authorSahin, Fahri
dc.date.accessioned2019-10-27T22:30:54Z
dc.date.available2019-10-27T22:30:54Z
dc.date.issued2015
dc.departmentEge Üniversitesien_US
dc.description.abstractParoxysmal nocturnal hemoglobinuria (PNH) is a rare, progressive, and life-threatening hematopoietic stem cell disorder characterized by complement-mediated intravascular hemolysis and a prothrombotic state. Patients with PNH might have slightly increased risk of infections due to complement-associated defects subsequent to CD59 deficiency. Here, we report a rare case of a 65-year-old male patient with necrotic ulcers on both legs, where the recognition of pancytopenia and microthrombi led to the diagnosis of PNH based on FLAER (FLuorescent AERolysin) flow cytometric analysis. He was subsequently started on eculizumab therapy, with starting and maintenance doses set as per drug labelling. Progression of the patient's leg ulcers during follow-up, with fulminant tissue destruction, purulent discharge, and necrotic patches, led to a later diagnosis of necrotizing fasciitis due to Pseudomonas aeruginosa and Klebsiella pneumonia infection. Courses of broad-spectrum antibiotics, surgical debridement, and superficial skin grafting were applied with successful effect during ongoing eculizumab therapy. This case highlights the point that it is important to maintain treatment of underlying disorders such as PNH in the presence of life-threatening infections like NF.en_US
dc.identifier.doi10.1155/2015/908087en_US
dc.identifier.issn2090-6560
dc.identifier.issn2090-6579
dc.identifier.pmid26347833en_US
dc.identifier.urihttps://doi.org/10.1155/2015/908087
dc.identifier.urihttps://hdl.handle.net/11454/51291
dc.identifier.wosWOS:000215217000071en_US
dc.identifier.wosqualityN/Aen_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherHindawi Ltden_US
dc.relation.ispartofCase Reports in Hematologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.titleNecrotizing Fasciitis in Paroxysmal Nocturnal Hemoglobinuriaen_US
dc.typeArticleen_US

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