Necrotizing Fasciitis in Paroxysmal Nocturnal Hemoglobinuria
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-associa...
Saved in:
| Main Authors: | , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2015-01-01
|
| Series: | Case Reports in Hematology |
| Online Access: | http://dx.doi.org/10.1155/2015/908087 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1832553658918633472 |
|---|---|
| author | Pusem Patir Yakup Isik Yigit Turk Mehmet Can Ugur Cengiz Ceylan Gulnur Gorgun Nihal Mete Gokmen Guray Saydam Fahri Sahin |
| author_facet | Pusem Patir Yakup Isik Yigit Turk Mehmet Can Ugur Cengiz Ceylan Gulnur Gorgun Nihal Mete Gokmen Guray Saydam Fahri Sahin |
| author_sort | Pusem Patir |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-0a60a2f87c394f7194378f9d43555d2a |
| institution | Kabale University |
| issn | 2090-6560 2090-6579 |
| language | English |
| publishDate | 2015-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Hematology |
| spelling | doaj-art-0a60a2f87c394f7194378f9d43555d2a2025-02-03T05:53:31ZengWileyCase Reports in Hematology2090-65602090-65792015-01-01201510.1155/2015/908087908087Necrotizing Fasciitis in Paroxysmal Nocturnal HemoglobinuriaPusem Patir0Yakup Isik1Yigit Turk2Mehmet Can Ugur3Cengiz Ceylan4Gulnur Gorgun5Nihal Mete Gokmen6Guray Saydam7Fahri Sahin8Department of Hematology, Ege University, 35100 Izmir, TurkeyDepartment of Plastic and Reconstructive Surgery, Ege University, 35100 Izmir, TurkeyDepartment of General Surgery, Ege University, 35100 Izmir, TurkeyDepartment of Internal Medicine, Tepecik Education and Training Hospital, Izmir, TurkeyDepartment of Internal Medicine, Tepecik Education and Training Hospital, Izmir, TurkeyDepartment of Internal Medicine, Tepecik Education and Training Hospital, Izmir, TurkeyDepartment of Immunology, Ege University, 35100 Izmir, TurkeyDepartment of Hematology, Ege University, 35100 Izmir, TurkeyDepartment of Hematology, Ege University, 35100 Izmir, TurkeyParoxysmal 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.http://dx.doi.org/10.1155/2015/908087 |
| spellingShingle | Pusem Patir Yakup Isik Yigit Turk Mehmet Can Ugur Cengiz Ceylan Gulnur Gorgun Nihal Mete Gokmen Guray Saydam Fahri Sahin Necrotizing Fasciitis in Paroxysmal Nocturnal Hemoglobinuria Case Reports in Hematology |
| title | Necrotizing Fasciitis in Paroxysmal Nocturnal Hemoglobinuria |
| title_full | Necrotizing Fasciitis in Paroxysmal Nocturnal Hemoglobinuria |
| title_fullStr | Necrotizing Fasciitis in Paroxysmal Nocturnal Hemoglobinuria |
| title_full_unstemmed | Necrotizing Fasciitis in Paroxysmal Nocturnal Hemoglobinuria |
| title_short | Necrotizing Fasciitis in Paroxysmal Nocturnal Hemoglobinuria |
| title_sort | necrotizing fasciitis in paroxysmal nocturnal hemoglobinuria |
| url | http://dx.doi.org/10.1155/2015/908087 |
| work_keys_str_mv | AT pusempatir necrotizingfasciitisinparoxysmalnocturnalhemoglobinuria AT yakupisik necrotizingfasciitisinparoxysmalnocturnalhemoglobinuria AT yigitturk necrotizingfasciitisinparoxysmalnocturnalhemoglobinuria AT mehmetcanugur necrotizingfasciitisinparoxysmalnocturnalhemoglobinuria AT cengizceylan necrotizingfasciitisinparoxysmalnocturnalhemoglobinuria AT gulnurgorgun necrotizingfasciitisinparoxysmalnocturnalhemoglobinuria AT nihalmetegokmen necrotizingfasciitisinparoxysmalnocturnalhemoglobinuria AT guraysaydam necrotizingfasciitisinparoxysmalnocturnalhemoglobinuria AT fahrisahin necrotizingfasciitisinparoxysmalnocturnalhemoglobinuria |