BACKGROUND: Combined factor V and VIII deficiency (CF5F8D) is a rare autosomal recessive disorder, with an estimated prevalence of about 1:100,000 in the Jewish population. Affected individuals have between 5 and 30% of normal levels of factor V and VIII, whereas the levels of other plasma proteins are not altered. This bleeding disorder has been treated by replacement therapy with plasma infusion and FVIII concentrate as source of factor V and VIII, respectively, depending on the severity of the haemorrhage. CASE REPORT: The individual with CF5F8D was a 22-year-old man with APTT of 51 s (control 33 s), PT of 27 s (control 12 s), factor V of 13%, and factor VIII of 21%. He had four untreatable carious teeth, including the maxillary first premolars and the mandibular first and second right molar. Factor VIII concentrate infusion began 12 h before the first dental extraction procedure and was continued in 8 h intervals. Moreover, 10 ml/kg of fresh frozen plasma were given for compensating factor V deficiency. No remarkable haemorrhage occurred during and after the procedures. The levels of factor V and VIII post procedures were 64 and 75%, respectively. CONCLUSION: It seems that plasma levels of 6075% for both factor V and VIII may be sufficient for major dental procedures.
BACKGROUND:Combined factor V and VIII deficiency (CF5F8D) is a rare autosomal recessive disorder, with an estimated prevalence of about 1:100,000 in the Jewish population. Affected individuals have between 5 and 30% of normal levels of factor V and VIII, whereas the levels of other plasma proteins are not altered. This bleeding disorder has been treated by replacement therapy with plasma infusion and FVIII concentrate as source of factor V and VIII, respectively, depending on the severity of the haemorrhage. CASE REPORT: The individual with CF5F8D was a 22-year-old man with APTT of 51 s (control 33 s), PT of 27 s (control 12 s), factor V of 13%, and factor VIII of 21%. He had four untreatable carious teeth, including the maxillary first premolars and the mandibular first and second right molar. Factor VIII concentrate infusion began 12 h before the first dental extraction procedure and was continued in 8 h intervals. Moreover, 10 ml/kg of fresh frozen plasma were given for compensating factor V deficiency. No remarkable haemorrhage occurred during and after the procedures. The levels of factor V and VIII post procedures were 64 and 75%, respectively. CONCLUSION: It seems that plasma levels of 6075% for both factor V and VIII may be sufficient for major dental procedures.
Authors: Roula A Farah; Philippe de Moerloose; Isabelle Bouchardy; Michael A Morris; Wadad Barakat; Alain E Sayad; Marguerite Neerman-Arbez Journal: Thromb Haemost Date: 2006-05 Impact factor: 5.249