Capellato M G, Lazzaro A R, Marafioti F, Polato G, Girolami A
Institute of Medical Semiotics, University of Padua, Medical School, Padova, Italy.
Haematologia (Budap). 1987;20(3):179-87.
In this study we present a new case of Factor XIII deficiency. The proposita, a 34 year old woman, showed a deficiency of both subunit a and subunit b, and a moderate bleeding tendency. Because of the concomitant decrease of subunits a and b the proposita is considered to be an example of Type I disease. Factor XIII levels were less than 10% both as activity and antigen. Several family members showed intermediate levels of both subunit a and b and were asymptomatic. They were considered to be heterozygotes. The hereditary pattern is autosomal incompletely recessive. Type I disease appears much less frequent than Type II.