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A study of VIIIC and VIIICAg in normals and hemophilia a patients in a brazilian population

Jorge E. quezada Díaz; Israel Roisenberg; Rivo R. Fischer

Departamento de Genética, Instituto de Biociências, Universidade Federal do Rio Grande do Sul, Caixa Postal 1953, 90001 Porto Alegre, RS, Brasil. Send correspondence to I.R.

ABSTRACT

Factor VIII clotting activity (VIIIC) was assayed by a one-stage technique and factor VIII clotting antigen (VIIICAg) by the inhibitor-neutralization technique in 40 unrelated normal persons (20 women and 20 men) and in 93 patients with hemophilia A belonging to 72 families. Among the hemophiliacs, the VIIIC/VIIICAg ratio permitted classifying 12 patients as A⁺ and 81 as A^-. In normals, there were no significant sex-related differences in mean VIIIC or VIIICAg levels.
A significant correlation between VIIIC and VIIICAg levels was observed in both normal and à patients but not in A⁺ patients. Mean VIIIC levels were similar for the two groups of patients but mean VIIICAg level was significantly higher in the A⁺ group.
The distribution of A⁺ and à types in families with a sporadic hemophiliac and in those with recurrent hemophilia was similar. Hemophilia type was concordant within all families in which more than one patient could be examined.

Keywords: VIIIC; VIIICAg; hemophilia.

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