Conflict of interest: Nothing to declare.
Prophylactic administration of prothrombin complex concentrates for congenital prothrombin deficiency with a novel frameshift mutation, prothrombin saitama†
Article first published online: 14 NOV 2012
Copyright © 2012 Wiley Periodicals, Inc.
Pediatric Blood & Cancer
Volume 60, Issue 3, pages 503–505, March 2013
How to Cite
Seki, M., Koh, K., Inoue, T., Tomita, Y., Kato, M., Shimizu, M., Morishita, E. and Hanada, R. (2013), Prophylactic administration of prothrombin complex concentrates for congenital prothrombin deficiency with a novel frameshift mutation, prothrombin saitama. Pediatr. Blood Cancer, 60: 503–505. doi: 10.1002/pbc.24387
- Issue published online: 15 JAN 2013
- Article first published online: 14 NOV 2012
- Manuscript Accepted: 9 OCT 2012
- Manuscript Received: 15 MAY 2012
- prophylactic therapy;
- prothrombin complex concentrates;
- prothrombin deficiency
Prothrombin (Factor II, FII) deficiency is an extremely rare autosomal recessive condition with an estimated incidence of 1:2 million. As severe and life-threatening bleeding is rare in FII deficiency, on demand therapy with administration of prothrombin complex concentrates (PCCs) or fresh frozen plasma is generally performed, and prophylactic therapy for FII deficiency has been reported in only three cases. Thus, its optimal dosage and schedule has remained uncertain. Here we report a case of severe prothrombin deficiency with a novel frameshift mutation of the F2 gene, who was started on prophylactic administration. Pediatr Blood Cancer 2013; 60: 503–505. © 2012 Wiley Periodicals, Inc.