Coagulation Studies in a Case of Severe Congenital Hypofibrinogenemia

T Barbui; P. I Porciello; E Dini

doi:10.1055/s-0038-1649048

Thrombosis and Haemostasis, Table of Contents

Thromb Haemost 1972; 28(01): 129-134
DOI: 10.1055/s-0038-1649048

Original Article

Schattauer GmbH

Coagulation Studies in a Case of Severe Congenital Hypofibrinogenemia

Authors

T Barbui

¹Division of Haematology (Director: Prof. E. Dini), Regional Civil Hospital, Vicenza, Italy, and Division of Cardiology (Director: Prof. A. Poppi), Regional Civil Hospital, Verona, Italy
P. I Porciello

¹Division of Haematology (Director: Prof. E. Dini), Regional Civil Hospital, Vicenza, Italy, and Division of Cardiology (Director: Prof. A. Poppi), Regional Civil Hospital, Verona, Italy
E Dini

¹Division of Haematology (Director: Prof. E. Dini), Regional Civil Hospital, Vicenza, Italy, and Division of Cardiology (Director: Prof. A. Poppi), Regional Civil Hospital, Verona, Italy

Abstract

Summary

A case of severe congenital hypofibrinogenemia is presented. The patient was a 26 year old female with congenital heart disease.

Partial thromboplastin time, Quick’s time and thrombin time were infinite. There was no immunoelectrophoretic precipitation with anti-fibrinogen serum. The thrombo-elastogram, carried out in PPP, indicated the complete lack of fibrinogen. On the contrary, the thromboelastographic pattern in PRP showed a very small opening of the branches, suddenly followed by their rejoining. Using an immunodiffusion method, it was possible to estimate the fibrinogen level at 12 mg%.

Full Text

References

References
1 Aznar J, Fernandez-Rego J. A, Fernandez Pla M. A, Aznar J. A. 1970; Congenital hypofìbrinogenemia in three members of a family. Coagulation 3: 279.
2 Caen J, Larrieu M. J, Samama M. 1968 L’hémostase. Méthodes d’exploration et diagnostic pratique, L’Expansion Editeur, Paris.
3 Hicks N. D, Pitney W. R. 1957; A rapid screening test for disorders of thromboplastin generation. British Journal Haematology 3: 227.
4 Miale J. B. 1967. Laboratory Medicine Haematology. The C. V. Mosby Company; Saint Louis:
5 Mustard J. F, Hegardt D, Rowsell H. C, McMillan R. L. 1964; Effect of adenosine nucleotides on platelet aggregation and clotting time. Journal of Laboratory and Clinical Medicine 64: 548.
6 O’Neill B.J, Hutton R. A. 1966; Prolonged bleeding time in congenital heart disease. Journal Clinical Pathology 19: 99.
7 Palumbo E, Dini E. 1956; Su di un nuovo metodo per il conteggio diretto delle piastrine. Haematologica 41: 373.
8 Parfentjev A, Johnson M. C, Clifton E. F. 1953; The determination of plasma fibrinogen by turbidity with ammonium sulphate. Archives of Biochemistry and Biophysics 46: 470.
9 Quick A. J. 1951. The physiology and pathology of hemostasis. Lea and Febiger; Philadelphia:
10 Regoeczi E. 1971; Iodine-labelled fibrinogen: A review. British Journal of Haematology 20: 649.
11 Revol L. 1962; Les grandes hypofibrinogénémies constitutionnelles hémorrhagiques. Hémostase 2: 243.
12 Salzman E. W. 1963; Measurement of platelet adhesiveness. A simple in vitro technique demonstrating an abnormality in von Willebrand’s disease, Journal of Laboratory and Clinical Medicine 62: 724.
13 Scheidegger J. J. 1955; Une microméthode de l’immunoelectrophorèse. International Archives Allergy 7: 103.