Eculizumab treatment for ischemic enteritis accompanied with paroxysmal nocturnal hemoglobinuria: a case report and literature review
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem cell disease characterized by intravascular hemolysis by complement as a result of clonal expansion of hematopoietic stem cells having acquired mutations in PIG-A gene. PNH is known to cause deep venous thrombosis and this accounts for a large part of the causes of death in this disease [1, 2]. Ischemic enteritis accompanied with PNH was life-threatening complications associated with extensive ischemia. We herein included a case report and literature review of treatments for ischemic enteritis with PNH.
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Conflicts of interest
Dr. Kurokawa reports grants and personal fees from Alexion Pharmaceuticals, Inc. and Asahi Kasei Pharma Corporation, and personal fees from Shionogi & Co., Ltd. outside the submitted work. The other authors declare that they have no conflict of interest.
Informed consent was obtained from the patient for being included in this case report.
- 1.Nishimura J, Kanakura Y, Ware RE, Shichishima T, Nakakuma H, Ninomiya H, Decastro CM, Hall S, Kanamaru A, Sullivan KM, Mizoguchi H, Omine M, Kinoshita T, Rosse WF (2004) Clinical course and flow cytometric analysis of paroxysmal nocturnal hemoglobinuria in the United States and Japan. Medicine (Baltimore) 83(3):193–207CrossRefGoogle Scholar
- 10.Quentin V, Dinasquet M, Rioux-Leclercq N, de Lajarte-Thirouard AS, Lotrian D, Lamy T, Bernard M, Olivié D, Gosselin M, Bretagne JF, Heresbach D (2003) Hémoglobinurie paroxystique nocturne avec ischémie digestive compliquée de perforation de l’intestin grêle. Gastroenterol Clin Biol 27(10):927–931PubMedGoogle Scholar