Inherited factor X deficiency: presentation of a case with etiologic and treatment considerations

The first documented case of inherited factor X deficiency in the dental literature is presented. Its ascertainment as a result of postoperative surgical complications illustrates the clinician's need to be familiar with the hereditary bleeding diatheses, as treatment is dependent on the underlying etiology of the specific disorder. In the present case treatment included administration of the antifibrinolytic agent epsilon-aminocaproic acid (EACA) and fresh frozen plasma. On the basis of our findings, a minimal therapeutic level of circulating factor X is estimated to be 15 percent of the normal level. Genetic heterogeneity within the factor X deficiency phenotype is discussed and, on the basis of laboratory findings, a CRM-positive autosomal recessive structural or regulator gene defect is proposed as the etiologic factor in the current case. Forty-nine cases in the literature are reviewed to delineate the pattern of bleeding in hereditary factor X deficiency.