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 delaye...

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Bibliographic Details
Published inThrombosis and haemostasis Vol. 42; no. 3; p. 994
Main Authors D'Souza, L, Coots, M C, Glueck, H I
Format Journal Article
LanguageEnglish
Published Germany 01.01.1979
Subjects
Amino Acids
Blood Coagulation Tests
Electrophoresis, Polyacrylamide Gel
Female
Fibrinogen - genetics
Fibrinogen - isolation & purification
Humans
Immunoelectrophoresis
Molecular Weight
Polymers
Pseudotumor Cerebri - blood
Pseudotumor Cerebri - complications
Thrombin Time
Thromboembolism - blood
Thromboembolism - complications
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Summary: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.
ISSN:0340-6245
DOI:10.1055/s-0038-1656990