Severe Hereditary Deficiency of Factor VII during Pregnancy

Summary

In normal pregnant women factor VII level is increased. This may also be observed in women with hereditary partial factor VII deficiency.

In a 38 year old woman with severe hereditary factor VII deficiency no change of the factor level was observed during pregnancy. The patient underwent two uneventful caesarian sections because of placenta praevia and transverse lie of the fetus.

During the second caesarian section factor VII level was simultaneously determined in blood obtained from the antecubital and uterine veins and from the umbilical vein and artery. Factor VII levels in the umbilical vessels were similar and exceeded the levels observed in the mother’s vessels. In 3 control patients similar examinations were performed during caesarian sections but reversed ratios of factor VII levels were observed. These data seem to prove that factor VII does not cross the placenta.

In the family study 2 siblings were found to have severe factor VII deficiency whereas several other members had either normal or partial deficient levels. The results obtained support again the assumption that factor VII deficiency is inherited by an autosomal gene that in the homozygous state is manifested by severe factor VII deficiency and in the heterozygous state by mild deficiency or normal factor VII levels.

• References

• 1 Loeliger A, Koller F. Behaviour of factor VII and prothrombin in late pregnancy and in the newborn. Actahaemat. (Basel) 07: 157-161 1952;
• 2 Alexander B, Meyers L, Kenny J, Goldstein R, Gurewich V, Grinspoon L. Blood coagulation in pregnancy. Proconvertin and prothrombin and the hypercoagulable state. New Engl. J. Med 254: 358-363 1956;
• 3 Owen C. A, Amundsen M. A, Thompson J. M, Spittell J. A, Bowie E. J. W, Stilwell G. G, Hewlet J. S, Mills S. D, Sauer W. G, Gage B. F. Congenital deficiency of factor VII (hypoconvertinemia). Critical review of literature and report of three cases with extensive pedigree study and effect of transfusion. Amer. J. Med 37: 71-91 1964;
• 4 Boiler L, Thompson J. M. Clotting factors during oral contraception: Further report. Brit, med. J 02: 23-25 1966;
• 5 Koller F. Importance of coagulopathies for genetics in general. Proceedings of the Seventh Congress of the International Society of Hematology. Vol. 1 170-175 Grüne & Stratton, Inc; New York: 1958
• 6 Hall C. A, Bapaport S. I, Ames S. B, Degroot J. A, with the technical assistance of Allen E. S, Ralston M. A. A Clinical and family study of hereditary proconvertin (factor VII) deficiency: Amer. J. Med 37: 172-181 1964;
• 7 Seligsohn U, Shani M, Ramot B, Adam A, Sheba C. h. Hereditary deficiency of Blood clotting factor VII and Dubin-Johnson syndrome in an Israeli family. Israel J. med. Sci 05: 1060-1065 1969;
• 8 Quick A. J. The physiology and pathology of Hemostasis. Lea & Febiger; Philadelphia: 1951: 125-134.
• 9 Owren B. A, Aas K. The control of dicumarol therapy and the quantitative determination of prothrombin and proconvertin. Scand. J. clin. Lab. Invest 03: 201-208 1951;
• 10 Pechet L. as quoted by Tocantins, L. M., L.A. Kazal in Blood Coagulation, Hemorrhage and Thrombosis. Methods of Study. 2nd ed.. Grune & Stratton; N. Y: 1964: 216-218.
• 11 Hardisty R. M, Ingram G. I. C. Bleeding Disorders: Investigation and Management. Blackwell Scientific Publications; Oxford: 1965: 307.
• 12 Denson K. W. The specific assay of Prower-Stuart factor and factor VII. Acta haemat. (Basel) 25: 105-120 1961;
• 13 Broctor R. R, Rapaport S. I. The partial thromboplastin time with koalin. A simple screening test for first stage plasma clotting factor déficiences. Amer. J. clin. Path 36: 212-219 1961;
• 14 Hardisty R. M, Ingram G. I. C. Bleeding Disorders: Investigation and management. Black - well Scientific Publications: Oxford; 1965: 285-287.
• 15 Tishkoff G. H, Bechet L, Alexander B. Some Biochemical and electrophoretic studies on purified prothrombin, factor VII (proconvertin) and factor X (Stuart). Blood 15: 778-790 1960;
• 16 Brydz H. Studies on proconvertin (factor VII). II Purification. Scand. J. clin. Lab. Invest 16: 101-107 1964;
• 17 Cade J. F, Hirsh J, Martin M. Placental barrier to coagulation factors: Its relevance to the coagulation defect at birth and to haemorrhage in the newborn. Brit. med. J 02: 281-283 1969;