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

Copyright © 2000 EDIZIONI MINERVA MEDICA

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


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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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