Menorrhagia due to congenital factor V deficiency in an adolescent

• Published in: Archives de pédiatrie : organe officiel de la Société française de pédiatrie 2013-01, Vol.20 (1), p.33-36
• Main Authors: Chemaou, A, Ayachi, M, Benjelloun, O, Zineddine, A
• Format: Article
• Language: fre
• Subjects: Adolescent; Factor V Deficiency - complications; Factor V Deficiency - diagnosis; Factor V Deficiency - therapy; Female; Humans; Menorrhagia - diagnosis; Menorrhagia - etiology; Menorrhagia - therapy; Plasma; Shock, Hemorrhagic - diagnosis; Shock, Hemorrhagic - etiology; Shock, Hemorrhagic - therapy; Treatment Outcome
• ISSN: 1769-664X
• DOI: 10.1016/j.arcped.2012.10.009

The frequency of menometrorrhagia in adolescence is estimated at 2-5 %. The functional origin is the most common. Hemostasis abnormalities account for 20 % of cases, and they should be sought whenever there is a personal history of known or suggestive of hemostasis disorders (epistaxis, gingival bleeding, postoperative bleeding, bruising, etc.) or there is a family history of hemostasis disorders. Organic origins are rare, but these must be sought when the characteristics of bleeding point in this direction. The workup aims to investigate the cause and assess the impact of the hemorrhage. It may include an NFS, blood group and rhesus±β-HCG, hemostasis evaluation, determination of hemostasis factors, and a pelvic ultrasound. Whatever the cause of menometrorrhagia, the therapeutic options are the same and are oriented by the severity of bleeding. The treatment is mainly medical and hormonal. The surgical option should be a choice of last resort. We report a case of a 13-year-old girl admitted in hemorrhagic shock due to abundant metrorrhagia. Her laboratory tests showed an isolated deficiency of factor V with a 2 % rate. Congenital factor V deficiency is a rare autosomal recessive coagulation disorder. It can be diagnosed at any age when a bleeding disorder of varying severity is observed. The diagnosis is based on the hemostasis evaluation with quantitative determination of factor V. Because no FV-specific concentrate is available, fresh frozen plasma remains the mainstay of treatment. Antifibrinolytics can also be beneficial, and platelet transfusions have been successfully used, associated with hormone therapy. These patients may best be managed in cooperation with both a gynecologist and a hematologist.