Severe factor XIII deficiency in pregnancy: A case report

Factor XIII (FXIII) deficiency is a rare inherited coagulation disorder that can cause recurrent pregnancy loss, placental complications and postpartum haemorrhage due to loss of clot stabilization. Managing pregnancy in affected women is challenging especially in low-resource settings where availability of FXIII concentrates and investigations for monitoring are limited. We report a case of severe congenital FXIII deficiency in a woman with four recurrent first-trimester pregnancy losses for whom diagnosis was confirmed by clot solubility testing and quantitative FXIII assays. Due to financial constraints, prophylactic cryoprecipitate was given instead of Factor XIII concentrate. Even in the absence of serial quantitative factor XIII levels, with multidisciplinary care, structured antenatal surveillance and planned peripartum FXIII replacement using cryoprecipitate, she could achieve two successful term deliveries without major maternal or neonatal bleeding complications. Thus, cryoprecipitate is an effective alternative when FXIII concentrate is unavailable.