A syndrome of factor VII deficiency and abnormal platelet release reaction.

A 15-year-old girl with severe factor VII deficiency and chronic arthropathy showed an excessively prolonged bleeding time. Further studies demonstrated low platelet adhesiveness and abnormal platelet aggregation with ADP, collagen and epinephrine. Release of 14C-serotonin was deficient after aggregation with ADP and epinephrine, but was normal with thrombin. Transfusion of plasma or prothrombin complex concentrate resulted in a partial or complete correction of the bleeding time, respectively, but had no effect on in vitro platelet function tests. Both parents and the only sister had factor VII activities of 42%-72% and factor VII antigen levels of 45%-66% of normal and may thus be heterozygotes with respect to factor VII deficiency. All three had normal bleeding times in spite of abnormal in vitro platelet functions. The observations are interpreted to mean that in this family with factor VII deficiency and abnormal platelet release reaction the platelet abnormality as such was not sufficiently severe to prolong the bleeding time unless the factor VII activity was also very low.

Effect of estrogen treatment on coagulation and fibrinolysis in postmenopausal women. With special reference to cold activation of factor VII.

Estriol (E3) and estradiol (E2) was given to 81 women with perimenopausal complaints. Venous blood was sampled before and after 2 weeks' treatment. In plasma coagulation and fibrinolysis the most marked estrogen effects were seen within the extrinsic coagulation system, with increased Thrombotest and Normotest activities. The incidence of cold activation of factor VII showed a marked increase after treatment with 6 mg of E2 or 8 mg of E3. The cold activation of factor VII was correlated to an activation of the plasma kallikrein system, as revealed by the peptidase activity of plasma kallikrein. The treatment with E2 and E3 induced changes also in the plasma proteolytic capacity, and in some proteinase inhibitors (antithrombin III, Cl inactivator). Like the effects within the plasma coagulation and kallikrein systems, these effects were similar to those reported after treatment with ethinyl estradiol, mestranol, and diethylstilbestrol, and in pregnancy. The conclusion is drawn, therefore, that the coagulation and fibrinolytic effects of E2 and E3 are basically similar to those of other estrogens, the differences between the various estrogens being more of a quantitative than of a qualitative nature. For the changes described, no absolute dose dependency could be found. Thus, treatment with E2 and E3 should be submitted to the same precautions as treatment with other estrogens.