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A description of two cases of factor V deficiency
Authors:FEDERICO CERNECA S.B.    ERGIO PARCO M.D.    ROBERT SIMEONE S.B.    BRUNO BEMBI M.D.   RITA GIORGI M.D.
Affiliation:Servizio Analisi Chimico-Cliniche, Laboratorio di Patologia dell'Emostasi Istituto per l'Infanzia 'Burlo Garofulo', via dell'Istria 65/1, 34137 Trieste, Italy.*;Servizio Analisi Chimico-Cliniche, Laboratorio di Patologia dell'Emostasi, Istituto per l'infanzia 'Burlo Garofolo'. Trieste, Italy.;Servizio Analisi Chimico-Cliniche, Laboratorio di Patologia dell'Emostasi, Istituto per l'Infanzia 'Burlo Garofolo', Trieste, Italy.;Pediatric Clinic, IRCSS Burlo Garofolo, Trieste, Italy.;Pediatric Clinic, IRCSS Burlo Garofolo, Trieste, Italy.
Abstract:
Summary We report two cases of factor V deficiency. A 12-year-old girl with thalassemia major was admitted for bone marrow transplant (mismatched). She was found to have a heterozygous deficiency of factor V (21%), but this was considered compatible with the transplant, which was performed without the support of suppernatant cryoprecipitate. A 14-year-old girl (factor V 2%) with a negative history (menarche at age 12, menstrual cycle regular and normal in quantity and length). One year previously she had complained of menorrhagia (length of period, 9 days); tests revealed server anaemia (Hb 4.9 g/dl). We show how even severe cases of factor V deficiency may often be silent, and not require transfusion in surgical procedures, thus avoiding the risks associated with such therapy.
Keywords:factor V    factor V deficiency    coagulation tests    parahaemophilia    menorrhagia    bone marrow transplant
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