Ersel DAĞ, Murat BULANIK, Zeynep ÖZCAN DAĞ, Demet DEMİRAY, Yavuz ŞİMŞEK

Cerebral Sinus Thrombosis During Pregnancy Associated with Protein S Deficiency: Report of a Rare Case

Herediter trombofilik mutasyonlar genel popülasyonun %10unda görülür ve bir çok olgu gebelik sırasında ortaya çıkan komplikasyonlar sonucunda tanı alır. Bunlardan Protein S eksikliği nadir görülen ve şiddetli trombojenik özellikte bir herediter trombofiliadır. Protein S eksikliği eşlik eden gebelikler genelde fetal kayıpla sonuçlanır. Bu yazıda canlı doğumla son bulan gebeliği sırasında, 30. gebelik haftasında serebral sinüs trombozu geçiren ve tetkiklerinde protein S eksikliği saptanan bir olgu sunularak konu literatür bilgileri eşliğinde tartışıldı.

Gebelite Protein-S Eksikliği İle İlişkili Serebral Sinüs Trombozu: Nadir Bir Olgu Sunumu

Abstract Hereditary thrombophylic mutations are seen in 10% of the general population and many cases are diagnosed as a result of complications that occur during pregnancy. Of these, Protein S deficiency is a rare hereditary trombophilia with severe thrombogenic features. Pregnancies accompanying protein S deficiency often result in loss of fetus. In this paper, we present the case of a protein S deficiency patient who eventually had a live birth despite the cerebral sinus thrombosis in the 30th week of her pregnancy and discuss the case with references to the relevant literature.

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1. Rezende SM, Simmonds RE, Lane DA. Coagulation, inflammation, and apoptosis: different roles for protein S and the protein S-C4b binding protein complex. Blood. 2004; 103(4):1192-201.
2. Buchanan GS, Rodgers GM, Ware Branch D. The inherited thrombophilias: genetics, epidemiology, and laboratory evaluation. Best Pract Res Clin Obstet Gynaecol. 2003;17(3):397-411.
3. Marlar RA, Neumann A. Neonatal purpura fulminans due to homozygous protein C or protein S deficiencies. Semin Thromb Hemost. 1990;16(4):299309.
4. Bowles L, Cohen H. Inherited thrombophilias and anticoagulation in pregnancy. Best Pract Res Clin Obstet Gynaecol. 2003;17(3):471489.
5. Haemostasis and Thrombosis Task Force, British Committee for Standards in Haematology. Investigation and management of heritable thrombophilia. Br J Haematol 2001;114(3):512-28.
6. Wypasek E, Undas A. Protein C and protein S deficiency - practical diagnostic issues. Adv Clin Exp Med. 2013;22(4):459-67.
7. Forestier F, Daffos F, Rainaut M, Solé Y, Amiral J. Vitamin K dependent proteins in fetal hemostasis at mid trimester of pregnancy. Thromb Haemost. 1985;24:53(3):401-3
8. 8. Alvis-Miranda HR, Milena Castellar-Leones S, Alcala-Cerra G, Rafael Moscote-Salazar L. Cerebral sinus venous thrombosis. J Neurosci Rural Pract. 2013;4(4):427-38.
9. Romualdi E, Dentali F, Rancan E, Squizzato A, Steidl L, Middeldorp S et al.. Anticoagulant therapy for venous thromboembolism during pregnancy: a systematic review and a meta-analysis of the literature. J Thromb Haemost. 2013;11(2):270-81.