Fibrinogen ''New York'--an abnormal fibrinogen associated with thromboembolism: functional evaluation
| Autor: | H, Al-Mondhiry, S B, Bilezikian, H L, Nossel |
|---|---|
| Rok vydání: | 1975 |
| Předmět: |
Immunodiffusion
Serotonin Platelet Aggregation Heparin Immunology Fibrinogen Cell Biology Hematology Hirudins Hydrogen-Ion Concentration Middle Aged Biochemistry Iodine Radioisotopes Aprotinin Postoperative Complications Thromboembolism Humans Female Blood Coagulation Tests Carbon Radioisotopes Warfarin Peptides Immunoelectrophoresis Mastectomy |
| Zdroj: | Blood. 45:607-619 |
| ISSN: | 1528-0020 0006-4971 |
| DOI: | 10.1182/blood.v45.5.607.bloodjournal455607 |
| Popis: | A 54-yr-old woman presented with a 23-yr history of repeated life- threatening thromboembolism. The presence of a qualitatively abnormal fibrinogen was suggested by the demonstration of delayed and incomplete coagulation of plasma or partially purified fibrinogen by thrombin or Reptilase. Two brothers showed a similar in vitro defect but were clinically not affected. The plasma fibrinogen concentration was 0.50- 1.64 mg/ml when estimated by heat turbidity, clottability, or immunologic techniques. The serum contained 80–820 mug/ml of unclottable fibrinogen-related materials even after 24 hr exposure to thrombin. The fibrinogen-related material in the serum showed faster anodal mobility an immunoelectrophoresis than that of normal plasma. Immunodiffusion studies with rabbit antihuman fibrinogen antiserum showed lines of identity between control plasma and the patient's plasma and serum. Studies of the kinetics of thrombin action on fibrinogen demonstrated impaired release of fibrinopeptide A and B and defective polymerization of preformed fibrin monomers. The maximum amount of fibrinopeptide A released by exhaustive treatment with thrombin was similar (per milligram protein) for both the patient's and control fibrinogen. This abnormal fibrinogen varient is tentatively designated fibrinogen “New York”; its possible identity with one of the previously described abnormal fibrinogens has not been excluded. |
| Databáze: | OpenAIRE |
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