[Severe congenital factor V deficiency: case report].
BACKGROUND
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.
Anikó
Marosi
M
Fovárosi Onkormányzat Heim Pál Gyermekkórház, Budapest, Hematológiai Osztály.
Eniko
Apjok
A
Edina
Magyarosy
M
Teréz
Szabó
S
Eva
Ajzner
A
D001780:Blood Coagulation Tests; D003937:Diagnosis, Differential; D005166:Factor V Deficiency / Q000097:blood / Q000151:congenital* / Q000175:diagnosis*; D006579:Heterozygote; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D010375:Pedigree