Gebelikte Koagülasyon Bozuklukları
Özet
Bu çalışma, gebelikte koagülasyon, antikoagülan ve fibrinolitik sistemlerin hemostatik dengeyi sağlamak için sinerjik çalışmasını ve bu süreçteki fizyolojik değişiklikleri incelemektedir. Gebelik, intrapartum ve postpartum kanamalara karşı koruma sağlayan protrombotik ve hipofibrinolitik bir yapıya sahiptir; bu dönemde von Willebrand Faktörü (vWF), fibrinojen ve bazı koagülasyon faktörleri artarken, protein S seviyeleri azalır. Von Willebrand Hastalığı (VWH) en sık görülen kalıtımsal kanama bozukluğudur ve tiplerine göre gebelikte farklı klinik yönetimler gerektirir. Faktör VIII ve IX eksikliklerinden kaynaklanan Hemofili A ve B taşıyıcısı kadınlarda, gebelik ve doğum süreçleri multidisipliner bir yaklaşım ve tersiyer merkezlerde takip gerektirmektedir. Nadir faktör eksiklikleri, fibrinojen ve kalıtımsal trombosit fonksiyon bozuklukları (Bernard Soulier Sendromu ve Glanzmann Trombastenisi) da kanama veya paradoksal tromboz riskleri nedeniyle özel profilaktik tedaviler ve replasmanlar içerir. Gebelikte en sık görülen trombositopeni nedeni gestasyonel trombositopeni olup benign seyrederken; Primer İmmün Trombositopenik Purpura (ITP) ile Trombotik Trombositopenik Purpura (TTP) ve Hemolitik Üremik Sendrom (HÜS) gibi trombotik mikroanjiopatiler yaşamı tehdit eden ve preeklampsi/HELLP ile karışabilen durumlardır. Bu hastalıkların ayırıcı tanısının hızla yapılması, plazmaferez veya hedefe yönelik ilaç tedavilerinin başlanması anne ve fetüs mortalitesini azaltmada kritik öneme sahiptir.
This study examines the synergistic functioning of coagulation, anticoagulant, and fibrinolytic systems to maintain hemostatic balance during pregnancy, alongside the associated physiological changes. Pregnancy exhibits a prothrombotic and hypofibrinolytic state that protects against intrapartum and postpartum hemorrhage; during this period, von Willebrand Factor (vWF), fibrinogen, and certain coagulation factors increase, while protein S levels decrease. Von Willebrand Disease (vWD) is the most common inherited bleeding disorder, requiring distinct clinical management during pregnancy depending on its type. Carriers of Hemophilia A and B, caused by factor VIII and IX deficiencies, require a multidisciplinary approach and monitoring in tertiary centers during pregnancy and delivery. Rare factor deficiencies, fibrinogen disorders, and inherited platelet function defects (Bernard Soulier Syndrome and Glanzmann Thrombasthenia) also involve specific prophylactic treatments and replacements due to risks of hemorrhage or paradoxical thrombosis. While gestational thrombocytopenia is the most common and benign cause of low platelets in pregnancy, conditions like Primary Immune Thrombocytopenic Purpura (ITP) and thrombotic microangiopathies, such as Thrombotic Thrombocytopenic Purpura (TTP) and Hemolytic Uremic Syndrome (HUS), are life-threatening and easily confused with preeclampsia/HELLP. Rapid differential diagnosis of these disorders and the initiation of plasmapheresis or targeted medical therapies are critical to reducing maternal and fetal mortality.
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