[Parahemophilia. Study in a family of 3 generations].
Three generations of a family with congenital deficit of factor V are reported. Those subjects showing symptoms were heterozygous. The transmission of the disease appears to be autosomal recessive. From a clinical standpoint, the patients suffered moderate haemorrhages, mostly affecting mucosae, which became severe on occasions, chiefly after labour. Isolated factor V rates did not correlate with the severity of bleeding. The different variants reported in the literature are commented. Transfusion of fresh-frozen plasma seems and adequate therapeutical approach for this disorder.
Hernández
M C
MC
Hospital Santa María Madre, Servicio de Hematología y Hemoterapia, Orense.
Vila
P
P
Alvarez
M
M
D005166:Factor V Deficiency / Q000235:genetics*; D006801:Humans; D008297:Male; D010375:Pedigree
Parahemofilia. Estudio en una familia de tres generaciones.