Necrotizing Fasciitis in Paroxysmal Nocturnal Hemoglobinuria. (11th August 2015)

• Record Type: Journal Article
• Authors: Patir, Pusem; Isik, Yakup; Turk, Yigit; Ugur, Mehmet Can; Ceylan, Cengiz; Gorgun, Gulnur; Mete Gokmen, Nihal; Saydam, Guray; Sahin, Fahri
• Other Names: Konstantopoulos Kostas Academic Editor.
• Is Part Of: Case reports in hematology. Volume 2015(2015)
• Publication Date: 2015-08-11
• Subjects: Hematology -- Periodicals; Hematology -- Case studies -- Periodicals; Blood -- Diseases -- Periodicals; Hematology; Hematologic Diseases; Blood -- Diseases; Hematology; Electronic journals; Periodicals; Case studies; Periodicals; Case Reports; Fulltext; Internet Resources; Periodicals; 616.15
• DOI: 10.1155/2015/908087
• Languages: English
• ISSNs: 2090-6560

Abstract

Abstract : Paroxysmal 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.