Introduction: Congential deficiency of factor V is a rare condition, transmitted in autosomal recessive way. Heterozygote patients generally have no symptoms, homozygotes present with spontaneous and postoperative bleedings. About one-half of patients are diagnosed in adulthood.
Methods: The presented case is a double heterozygote, which was confirmed using moleculare biological methods. The authors found many carriers in his family.
Conclusions: This rare and sometimes severe disorder needs regular haematological controls. Carriers must have more attention during surgical procedures and labour, and the family doctor should be informed about there condition.
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