Recombinant FVIIa therapy for heavy menstrual bleeding in patients with severe FV deficiency

Allogeneic stem cell transplantation in a child with thalassemia major and severe factor V deficiency: Case report and review of the literature

“…The thalassemia major accompanying severe FV deficiency was a very rare condition, and complete chimerism was obtained for thalassemia major after allo-SCT, but stem cell transplant did not work to correct clotting abnormalities in our case. However, approximately 80% of FV was produced in the liver and the remaining 20% was stored in the alpha granule of megakaryocytes [3]. Thus, we were expected to increase in FV level after platelet engraftment due to the platelet-derived FV in allo-SCT, but we did not observe any changes in prolonged PT/ APTT with low FV level after allo-SCT.…”

“…In contrast to thalassemia, severe factor V deficiency (FV) is a rare inherited coagulation disorder in the world. Fresh frozen plasma is used for serious bleeding episodes because specific factor concentrate has not existed for patients with severe FV deficiency yet [3]. According to the literature search, simultaneous occurrences of thalassemia and FV deficiency have been reported in only three cases to date [4,5].…”

Clotting Factor Deficiencies as an Underlying Cause of Abnormal Uterine Bleeding in Women of Reproductive Age: A Literature Review

“…Although FV concentrate is not currently commercially available, an in vitro study has shown its ability to fully normalize both PT and aPTT, as well as thrombin generation parameters. For women with severe FV deficiency and HMB that is refractory to standard treatment, Kaya et al have proposed the administration of recombinant Factor VIIa (rFVIIa) as a potential therapeutic option [ 28 ].…”