Paroxysmal nocturnal hemoglobinuria: a red clot syndrome.
Ann Vasc Surg
; 28(1): 122.e5-10, 2014 Jan.
Article
in En
| MEDLINE
| ID: mdl-24200143
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.
Full text:
1
Database:
MEDLINE
Main subject:
Saphenous Vein
/
Femoral Artery
/
Peripheral Arterial Disease
/
Vascular Grafting
/
Hemoglobinuria, Paroxysmal
Type of study:
Diagnostic_studies
/
Etiology_studies
/
Risk_factors_studies
Language:
En
Year:
2014
Type:
Article