Article Type : Case Report
Title : Pregnancy Outcomes in a Rare Case of Combined Protein C and Protein S Deficiency: A Case Report and Review of Management Strategies
Abstract : Protein C and Protein S deficiencies are rare inherited thrombophilic disorders that increase the risk of venous thromboembolism (VTE) and pregnancy-related complications. The occurrence of combined Protein C and Protein S deficiency is exceedingly rare and poses a significant risk for adverse pregnancy outcomes. We report a case of a 28-year-old primigravida diagnosed with combined Protein C and Protein S deficiency who successfully carried her pregnancy to term under a carefully monitored anticoagulation regimen. Despite the high risk of thrombotic complications, early diagnosis and management allowed for a favorable maternal and fetal outcome. This case highlights the importance of multidisciplinary care in managing high-risk pregnancies with thrombophilic disorders.
Introduction : Thrombophilic disorders significantly impact pregnancy, increasing the risk of complications such as recurrent pregnancy loss, intrauterine growth restriction, preeclampsia, and venous thromboembolism (VTE)[1]. Protein C and Protein S deficiencies are rare inherited conditions associated with hypercoagulability [2]. Combined deficiency of both proteins is exceptionally uncommon, with limited literature on pregnancy outcomes in affected individuals [3-6]. This case report presents the successful management of a pregnancy in a woman with combined Protein C and Protein S deficiency, emphasizing the role of early detection and appropriate anticoagulation therapy.
Case Presentation : A 28-year-old primigravida with no prior history of thromboembolism presented at 10 weeks of gestation with a known family history of thrombophilia. She was asymptomatic but had been diagnosed with combined Protein C and Protein S deficiency during preconception screening. Laboratory tests confirmed significantly reduced Protein C (25%, reference range 70-140%) and Protein S (22%, reference range 60-130%) activity levels.
Given her high risk for thromboembolic events, she was initiated on prophylactic low-molecular-weight heparin (LMWH) at a therapeutic dose. Throughout her pregnancy, she was closely monitored for signs of thrombosis, placental insufficiency, and fetal growth restriction through serial ultrasonography and Doppler studies. Despite the inherent risks, she remained asymptomatic with stable fetal growth parameters.
At 38 weeks of gestation, she underwent an elective induction of labor under a controlled anticoagulation regimen. She delivered a healthy male infant weighing 3.1 kg via vaginal delivery. Postpartum, she was maintained on LMWH for six weeks to prevent postpartum VTE. The mother and infant remained in good health at follow-up.
Discussion : Combined Protein C and Protein S deficiency significantly elevates the risk of thromboembolic complications in pregnancy [7]. Pregnancy itself is a hypercoagulable state due to increased coagulation factors and decreased fibrinolysis, making anticoagulation therapy crucial in affected individuals [8]. LMWH remains the preferred anticoagulant due to its safety profile during pregnancy [9-10].
This case underscores the importance of individualized anticoagulation regimens and multidisciplinary management involving obstetricians, hematologists, and maternal-fetal medicine specialists. The successful pregnancy outcome in our patient was achieved through early diagnosis, vigilant monitoring, and adherence to an optimized anticoagulation strategy.
Pregnancy in women with combined Protein C and Protein S deficiency presents significant risks, but with proper anticoagulation therapy and multidisciplinary care, favorable maternal and fetal outcomes can be achieved. Awareness of this rare condition and its implications in pregnancy is crucial for timely intervention and risk minimization.
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