Fibrinogen Irvine: a qualitatively abnormal fibrinogen associated with the predisposition to recurrent visceral and peripheral venous thrombosis.
A slow clotting dysfibrinogen with delayed anodal immunoelectrophoretic mobility and impaired fibrinopeptide A release has been identified in a patient with recurrent portal vein and deep venous thrombosis. Affected family members tested in the initial screening were asymptomatic. The proband's father died of pulmonary embolism at age 44 years and had mesenteric thrombosis at necropsy. The association of a plasma protein abnormality with visceral thrombosis is unusual and has never been observed previously with a dysfibrinogen. The qualitative abnormality is transmitted as an autosomal codominant and is tentatively designated, fibrinogen Irvine.
D000328:Adult; D001778:Blood Coagulation Disorders / Q000097:blood*; D005340:Fibrinogen / Q000235:genetics / Q000302:isolation & purification*; D015241:Fibrinogens, Abnormal; D006801:Humans; D008297:Male; D010375:Pedigree; D012008:Recurrence; D013924:Thrombophlebitis / Q000097:blood*
D015241:Fibrinogens, Abnormal; C046595:fibrinogen Irvine; D005340:Fibrinogen