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Case Reports in Hematology
Volume 2015 (2015), Article ID 908087, 5 pages
http://dx.doi.org/10.1155/2015/908087
Case Report

Necrotizing Fasciitis in Paroxysmal Nocturnal Hemoglobinuria

1Department of Hematology, Ege University, 35100 Izmir, Turkey
2Department of Plastic and Reconstructive Surgery, Ege University, 35100 Izmir, Turkey
3Department of General Surgery, Ege University, 35100 Izmir, Turkey
4Department of Internal Medicine, Tepecik Education and Training Hospital, Izmir, Turkey
5Department of Immunology, Ege University, 35100 Izmir, Turkey

Received 14 June 2015; Revised 2 August 2015; Accepted 3 August 2015

Academic Editor: Kostas Konstantopoulos

Copyright © 2015 Pusem Patir et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.