title: Paroxysmal nocturnal hemoglobinuria: a red clot syndrome.
abstract: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, nonmalignant disorder of hematopoietic stem cells characterized by hemolysis, diminished hematopoiesis, and thrombophilia. We describe a 65-year-old woman with known PNH and peripheral arterial disease who presented with critical limb ischemia and a nonhealing left foot ulcer. She underwent surgical bypass of a diffusely diseased left superficial femoral artery with autologous reversed saphenous vein graft. Her postoperative course was complicated by wound sepsis and PNH exacerbation with resultant graft thrombosis requiring an above-knee amputation. This case highlights several key concepts relevant to the management of vascular surgery patients with PNH: (1) their predisposition for arterial and venous thrombosis; (2) hypercoagulability despite standard anticoagulation regimens; (3) the role of eculizumab (a monoclonal antibody that inhibits complement activation used to treat PNH) in reducing thrombotic complications and hemolysis; and (4) complications associated with the immunosuppressive effects of eculizumab. We recommend careful monitoring of hemolysis and immunosuppression, aggressive anticoagulation, frequent graft surveillance, and early consultation with hematology.
authors.affiliation: Division of Vascular Surgery, Department of Surgery, Oregon Health and Science University, Portland, OR.
authors.forename: Jeffrey D
authors.initials: JD
authors.lastname: Crawford
authors.affiliation: Division of Vascular Surgery, Department of Surgery, Oregon Health and Science University, Portland, OR.
authors.forename: Victor W
authors.initials: VW
authors.lastname: Wong
authors.affiliation: Division of Hematology and Oncology, the Department of Internal Medicine, Oregon Health and Science University, Portland, OR.
authors.forename: Thomas G
authors.initials: TG
authors.lastname: Deloughery
authors.affiliation: Division of Vascular Surgery, Department of Surgery, Oregon Health and Science University, Portland, OR.
authors.forename: Erica L
authors.initials: EL
authors.lastname: Mitchell
authors.affiliation: Division of Vascular Surgery, Department of Surgery, Oregon Health and Science University, Portland, OR.
authors.forename: Timothy K
authors.initials: TK
authors.lastname: Liem
authors.affiliation: Division of Vascular Surgery, Department of Surgery, Oregon Health and Science University, Portland, OR.
authors.forename: Gregory J
authors.initials: GJ
authors.lastname: Landry
authors.affiliation: Division of Vascular Surgery, Department of Surgery, Oregon Health and Science University, Portland, OR.
authors.forename: Amir F
authors.initials: AF
authors.lastname: Azarbal
authors.affiliation: Division of Vascular Surgery, Department of Surgery, Oregon Health and Science University, Portland, OR. Electronic address: monetag@ohsu.edu.
authors.forename: Gregory L
authors.initials: GL
authors.lastname: Moneta
mesh_terms: D000368:Aged; D000671:Amputation, Surgical; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D000925:Anticoagulants / Q000627:therapeutic use; D001777:Blood Coagulation / Q000187:drug effects; D016638:Critical Illness; D005260:Female; D005263:Femoral Artery / Q000000981:diagnostic imaging / Q000601:surgery*; D016523:Foot Ulcer / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D006083:Graft Occlusion, Vascular / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D006457:Hemoglobinuria, Paroxysmal / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D006461:Hemolysis / Q000187:drug effects; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007511:Ischemia / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D058729:Peripheral Arterial Disease / Q000097:blood / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D011859:Radiography; D012086:Reoperation; D012307:Risk Factors; D012501:Saphenous Vein / Q000637:transplantation*; D013530:Surgical Wound Infection / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D058017:Vascular Grafting / Q000009:adverse effects*; D020246:Venous Thrombosis / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D014945:Wound Healing
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D000925:Anticoagulants; D007166:Immunosuppressive Agents; C481642:eculizumab