Cryoprecipitate Meets Congenital Factor XIII Crisis: A Rare Case Report in Pregnancy

Abstract

Factor XIII deficiency is an extremely rare autosomal recessive bleeding disorder, with an incidence of 1 in 2-3 million live births. It is unique among coagulation factor deficiencies due to its association with recurrent miscarriage, impaired wound healing and life-threatening hemorrhage. Factor XIII (FXIII) is essential for coagulation, wound healing, angiogenesis, and maintenance of hemostatic milieu in pregnancy.   Diagnosis often relies on specific FXIII assays as routine tests may be normal. Rarely an acquired deficiency of Factor XIII has been described, that occurs secondary to either hyperconsumption (surgery, DIC, Sepsis, Thrombosis) or hypo-synthesis (liver disease, leukemia) or an immune-mediated process (SLE, RA, Malignancy). We hereby report a rare case of a pregnant woman with Congenital Factor XIII deficiency managed with cryoprecipitate therapy, who successfully carried her pregnancy to term and delivered a healthy baby. The case highlights the critical role of vigilant antenatal monitoring, timely replacement therapy with multidisciplinary co-ordination.