A Rare Case of Dysfibrinogenemia in Pregnancy: Management Decisions When Data Are Lacking

Congenital fibrinogen disorders are a rare group of inherited conditions characterized by quantitative and/or qualitative fibrinogen deficiencies.Dysfibrinogenemia is the most common of these disorders and can be diagnosed by standard coagulation blood testing and is often confirmed with pedigree and molecular genetic analyses.Because of variability in genotype-to-phenotype expression and the inadvertent combining of fibrinogen deficiency subtypes in the literature, the optimal management of these individuals in general, and particularly in pregnancy, remains incompletely understood and empirically managed.The authors present a case of a pregnant patient with well-defined congenital dysfibrinogenemia who was managed with laboratory monitoring and expectant care and had a favorable outcome.The authors review the current literature and discuss alternative approaches to care using new diagnostic screening methodologies.Management was guided by a multidisciplinary team, including maternal-fetal medicine, anesthesiology, pediatric, and adult hematologists.