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Panminerva Medica 1998 September;40(3):226-38

lingua: Inglese

Genetic defi­cien­cy of fac­tor VII and hemor­rhag­ic diath­e­sis

Iannello S., Prestipino M., Belfiore F.

From the Institute of Internal Medicine and Internal Specialties, Chair of Internal Medicine University of Catania Medical School, Garibaldi Hospital, cata­nia, Italy


FVII defi­cien­cy is a rath­er rare inher­it­ed hemo­coag­u­la­tion dis­or­der that pre­dis­pos­es to hemor­rhag­ic ­events, espe­cial­ly from ­mucous mem­branes, that are not pre­dict­able and ­severe as in hemo­phil­ia A. This ­defect pro­duc­es pro­longed pro­throm­bin time (PT), ­reduced activ­ity of FVII and nor­mal acti­vat­ed par­tial throm­bo­plas­tin time (aPTT). We ­report the case of a 43-year-old obese woman with ­severe defi­cien­cy of fac­tor VII (FVII), prob­ably genet­ic in ­nature, and meno-metror­rha­gia asso­ciat­ed with mul­ti­ple fibro­mas of uter­us. Our ­patient had no his­to­ry of bleed­ing in infan­cy and young age, and in the past, ­before the dis­ease was diag­nosed, under­went major sur­gery oper­a­tions (thy­roi­dec­to­my and cae­sar­ian sec­tion) with­out hem­or­rhage. Patient’s rel­a­tives with mild het­er­o­zy­gous defi­cien­cy of FVII (the ­father, a broth­er, a sis­ter, a ­sister’s daugh­ter and the ­patient’s son) did not show any bleed­ing ten­den­cy. This case ­report is dis­cussed in the light of lit­er­a­ture data ((source: Medline from 1964 to 1996). The dif­fer­ent forms of con­gen­i­tal (iso­lat­ed or com­bined with other clot­ting dis­or­ders) and ­acquired FVII defi­cien­cy, with the appro­pri­ate ther­a­pies, are ­reviewed. The cli­ni­cian must con­sid­er FVII defi­cien­cy in cases of recur­rent bleed­ing, and this dis­ease, even if rath­er rare, ­should not be under­es­ti­mat­ed in clin­i­cal prac­tice ­because it is poten­tial­ly fatal.

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