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[Parahemophilia. Study in a family of 3 generations]
M C Hernández1, P Vila, M Alvarez
1Hospital Santa María Madre, Servicio de Hematología y Hemoterapia, Orense.
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.
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.