An acquired abnormal fibrinogen associated with thromboembolic disease and pseudotumor cerebri.
An abnormal fibrinogen was found in a patient associated with disabling recurrent phlebitis and pulmonary emboli, pseudotumor cerebri, gout and endometriosis. The fibrinogen is characterized by (1) abnormal side-to-side and end-to-end polymerization, (2) abnormal fibrinopeptide release, (3) a delayed gamma-gamma dimerization of the non cross-linked fibrin, (4) a pH optimum of 7--7.8, and (5) a deviation from normal amino acid composition with regard to lysine, aspartic acid, glutamic acid and serine. Since no defect has been found in any of her three children, and since the prothromin and partial thromboplastin times vary from time to time, it is assumed that the defect is acquired. Liver disease, usually associated with acquired abnormal fibrinogen, has been excluded as an etiological cause since liver function tests and biopsy are completely normal.
D000596:Amino Acids; D001780:Blood Coagulation Tests; D004591:Electrophoresis, Polyacrylamide Gel; D005260:Female; D005340:Fibrinogen / Q000235:genetics / Q000302:isolation & purification; D006801:Humans; D007122:Immunoelectrophoresis; D008970:Molecular Weight; D011108:Polymers; D011559:Pseudotumor Cerebri / Q000097:blood* / Q000150:complications; D013918:Thrombin Time; D013923:Thromboembolism / Q000097:blood* / Q000150:complications
D000596:Amino Acids; D011108:Polymers; D005340:Fibrinogen