Table 1.

Coagulation parameters, plasma clot optical density, and results of DNA analysis for fibrinogen Paris V

Family member*PTT (sec)PT (sec) TT (sec) RT (sec)Fibrinogen (mg/dL) Paris V mutationΔOD 405 nm
Clauss Antigen
Normal  25-38 11-15  19-23  18.5-23  150-350   Negative 0.60 ± 0.11  
II.5  46.1  37.5 30  53  388 420  Positive 0.04  
III.1  34.9  12.7  30 57  258  314  Positive  0.13  
III.2  29.3 13.1  23  44  268  274  Positive  0.06  
III.3 28.8  13.4  29  50  190  186  Positive −0.02  
III.4  30.7  13.9  34  43  258  Positive  
III.11  44.3  30.2 30  50  306 406  Positive  −0.02  
IV.1  30.9  12.8  29 50  197  188  Positive  0.02  
IV.2  25.3  12.6 22  22  283  272  Negative  0.67  
IV.31-153       Positive  
IV.5  30.0  13.3  20 30  182  186  Positive  0.13  
IV.6  31.5  13.4 20  20  232  232  Negative  0.50  
IV.8  28.4 13.8  20  20  314  272  Negative  0.59  
IV.10 29.6  14.5  36  42  211   Positive  
IV.13 35.4  24.9 37  49  227  244  Positive 0.00 
Family member*PTT (sec)PT (sec) TT (sec) RT (sec)Fibrinogen (mg/dL) Paris V mutationΔOD 405 nm
Clauss Antigen
Normal  25-38 11-15  19-23  18.5-23  150-350   Negative 0.60 ± 0.11  
II.5  46.1  37.5 30  53  388 420  Positive 0.04  
III.1  34.9  12.7  30 57  258  314  Positive  0.13  
III.2  29.3 13.1  23  44  268  274  Positive  0.06  
III.3 28.8  13.4  29  50  190  186  Positive −0.02  
III.4  30.7  13.9  34  43  258  Positive  
III.11  44.3  30.2 30  50  306 406  Positive  −0.02  
IV.1  30.9  12.8  29 50  197  188  Positive  0.02  
IV.2  25.3  12.6 22  22  283  272  Negative  0.67  
IV.31-153       Positive  
IV.5  30.0  13.3  20 30  182  186  Positive  0.13  
IV.6  31.5  13.4 20  20  232  232  Negative  0.50  
IV.8  28.4 13.8  20  20  314  272  Negative  0.59  
IV.10 29.6  14.5  36  42  211   Positive  
IV.13 35.4  24.9 37  49  227  244  Positive 0.00 

PTT, partial thromboplastin time; PT, prothrombin time; TT, thrombin time; RT, reptilase time.

*

Numeric designation for family members is derived from the pedigree in Figure 1.

Indicates that the prothrombin time is prolonged because the patient was on warfarin.

Positive indicates that the patient is heterozygous for the fibrinogen Paris V mutation.

F1-153

Patient IV.3 died before plasma could be obtained for determining coagulation parameters. A tube of frozen whole blood was available for DNA extraction. He is not a carrier of the factor V Leiden or prothrombin gene 20210 G → A polymorphisms.

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