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Digital ischemia and gangrene due to red blood cell aggregation induced by acquired dysfibrinogenemia

H C Kwaan1, M Levin, S Sakurai

  • 1Department of Medicine, Northwestern University Medical School, Chicago, IL, USA.

Journal of Vascular Surgery
|January 10, 1998
PubMed

Digital gangrene was observed in a patient who had angiographic findings of digital arterial occlusion. The patient's blood showed a marked red blood cell aggregation with rouleaux formation in long chains, which could not be dispersed at shear rates up to 200 sec-1. Studies of the patient's blood revealed the presence of an abnormal fibrinogen capable of aggregating normal red blood cells. This fibrinogen was found by Raman spectroscopy to have an increased alpha-helical content, whereas the beta-sheet content was decreased. Defibrinogenation therapy with ancrod resulted in a dramatic symptomatic relief. The disappearance of the abnormal fibrinogen 6 months later and an absence of a family history indicate that this dysfibrinogenemia was acquired.

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