Paroxysmal nocturnal hemoglobinuria: A red clot syndrome

Jeffrey D. Crawford, Victor W. Wong, Thomas Deloughery, Erica Mitchell, Timothy Liem, Gregory Landry, Amir Azarbal, Gregory (Greg) Moneta

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
JournalAnnals of Vascular Surgery
Volume28
Issue number1
DOIs
StatePublished - Jan 2014

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Paroxysmal Hemoglobinuria
Hemolysis
Thrombophilia
Transplants
Foot Ulcer
Complement Activation
Peripheral Arterial Disease
Saphenous Vein
Hematopoiesis
Hematology
Femoral Artery
Immunosuppressive Agents
Hematopoietic Stem Cells
Amputation
Venous Thrombosis
Immunosuppression
Blood Vessels
Knee
Sepsis
Thrombosis

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery

Cite this

Paroxysmal nocturnal hemoglobinuria : A red clot syndrome. / Crawford, Jeffrey D.; Wong, Victor W.; Deloughery, Thomas; Mitchell, Erica; Liem, Timothy; Landry, Gregory; Azarbal, Amir; Moneta, Gregory (Greg).

In: Annals of Vascular Surgery, Vol. 28, No. 1, 01.2014.

Research output: Contribution to journalArticle

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