DIGITAL ISCHEMIA AND GANGRENE DUE TO RED-BLOOD-CELL AGGREGATION INDUCED BY ACQUIRED DYSFIBRINOGENEMIA

Digital gangrene was observed in a patient who had angiographic findin gs of digital arterial occlusion. The patient's blood showed a marked red blood cell aggregation with rouleaux formation in long chains, whi ch 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 c apable of aggregating normal red blood cells. This fibrinogen was foun d by Raman spectroscopy to have an increased alpha-helical content, wh ereas the beta-sheet content was decreased. Defibrinogenation therapy with ancrod resulted in a dramatic symptomatic relief. The disappearan ce of the abnormal fibrinogen 6 months later and an absence of a famil y history indicate that this dysfibrinogenemia was acquired.