Necrotizing Fasciitis in Paroxysmal Nocturnal Hemoglobinuria
Joint Authors
Patir, Pusem
Isik, Yakup
Turk, Yigit
Ugur, Mehmet Can
Ceylan, Cengiz
Gorgun, Gulnur
Mete Gokmen, Nihal
Saydam, Guray
Sahin, Fahri
Source
Issue
Vol. 2015, Issue 2015 (31 Dec. 2015), pp.1-5, 5 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2015-08-11
Country of Publication
Egypt
No. of Pages
5
Main Subjects
Abstract EN
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.
American Psychological Association (APA)
Patir, Pusem& Isik, Yakup& Turk, Yigit& Ugur, Mehmet Can& Ceylan, Cengiz& Gorgun, Gulnur…[et al.]. 2015. Necrotizing Fasciitis in Paroxysmal Nocturnal Hemoglobinuria. Case Reports in Hematology،Vol. 2015, no. 2015, pp.1-5.
https://search.emarefa.net/detail/BIM-1058647
Modern Language Association (MLA)
Patir, Pusem…[et al.]. Necrotizing Fasciitis in Paroxysmal Nocturnal Hemoglobinuria. Case Reports in Hematology No. 2015 (2015), pp.1-5.
https://search.emarefa.net/detail/BIM-1058647
American Medical Association (AMA)
Patir, Pusem& Isik, Yakup& Turk, Yigit& Ugur, Mehmet Can& Ceylan, Cengiz& Gorgun, Gulnur…[et al.]. Necrotizing Fasciitis in Paroxysmal Nocturnal Hemoglobinuria. Case Reports in Hematology. 2015. Vol. 2015, no. 2015, pp.1-5.
https://search.emarefa.net/detail/BIM-1058647
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1058647