Transfüzyon Bağımlı β-Talasemili Kadınlarda Gebelik Yönetimi: Klinik Önerilerle Kanıta Dayalı Derleme

Özet (abstract)

Abstract Transfusion-dependent β-thalassemia (TDT) is a severe hereditary hemoglobin disorder requiring lifelong regular blood transfusions, which inevitably result in iron overload and multiple organ complications. Advances in transfusion therapy, iron chelation, and multidisciplinary care have significantly improved survival and reproductive potential in women with TDT. Consequently, pregnancy has become increasingly feasible, although it remains a high-risk condition requiring specialized management. This narrative review summarizes the current evidence and international recommendations regarding pregnancy management in women with TDT. Relevant publications, including clinical practice guidelines, systematic reviews, meta-analyses, and original studies, were reviewed to identify evidence on preconception counseling, maternal assessment, antenatal care, transfusion strategies, iron chelation, fetal surveillance, delivery, and postpartum management. Forty evidence-based clinical recommendations were synthesized from current international guidelines and available literature. Successful pregnancy in women with TDT requires comprehensive preconception evaluation by a multidisciplinary team, including assessment of cardiac function, iron overload, endocrine disorders, liver disease, renal function, fertility, and genetic risk. During pregnancy, maintenance of pre-transfusion hemoglobin levels ≥ 10 g/dL, regular fetal growth surveillance, periodic cardiac assessment, screening for gestational diabetes, and individualized thromboprophylaxis in high-risk patients are recommended. Iron chelation therapy should generally be discontinued after conception. However, deferoxamine (DFO) may be considered from the second trimester onward in selected women with severe cardiac or hepatic iron overload; in women with significant myocardial iron, DFO has been used from approximately 20 weeks of gestation or earlier when clinically indicated. Appropriate management before and throughout pregnancy substantially reduces maternal and fetal complications. Although pregnancy in women with TDT is considered high risk, favorable maternal and fetal outcomes can be achieved through careful preconception planning, multidisciplinary management, regular monitoring, and adherence to evidence-based clinical recommendations. Early identification and treatment of iron overload, cardiac disease, endocrine dysfunction, and other comorbidities remain essential for optimizing pregnancy outcomes.

Yazarların özeti; kaynağından alınmıştır. Annals of Hematology, 2026 · DOI ↗