The rare inherited coagulation disorders.

University of Manchester, Manchester Blood Centre, Plymouth Grove, Manchester, UK. paula.bolton-maggs@manchester.ac.uk

Pediatric blood & cancer 2013

The rare inherited coagulation disorders (RICD) are uncommon and thus not well-defined in terms of severity or management. Inheritance is autosomal; in some of these disorders in the heterozygote state affected individuals may be mildly symptomatic. Severe deficiencies are more common in association with consanguinity. Factor X and factor XIII deficiency have the most severe manifestations, while factor XI deficiency is the least severe. Factor VII and factor XI deficiencies show a poor relationship between the factor level and bleeding risk. Unlike hemophilia, women are equally affected by these RICD and can have problems related to menstruation and childbirth. Copyright © 2012 Wiley Periodicals, Inc.