Pregnancy in Thalassemia

Abstract

Therapeutic advances, including the availability of oral iron chelators and new non-invasive methods for early detection and treatment of iron overload, have significantly improved the life expectancy and quality of thalassemia patients, with a consequent increase in their reproductive potential and desire to have children. Hundreds of pregnancies have been reported so far, highlighting that women carefully managed in the preconception phase usually carry out a successful gestation and labor, both in case of spontaneous conception and assisted reproductive techniques. A multidisciplinary team including a cardiologist, an endocrinologist, and a gynecologist, under the supervision of an expert in beta-thalassemia, should be involved. During pregnancy, a close follow-up of maternal disorders and of the baby's status is recommended. Hemoglobin should be maintained over 10 g/dL to allow normal fetal growth. Chelators are not recommended; nevertheless, it may be reasonable to consider restarting chelation therapy with desferrioxamine towards the end of the second trimester when the potential benefits outweigh the potential fetal risk. Women with non-transfusion-dependent thalassemia who have never previously been transfused or who have received only minimal transfusion therapy are at risk of severe alloimmune anemia if blood transfusions are required during pregnancy. Since pregnancy increases the risk of thrombosis three-fold to four-fold and thalassemia is also a hypercoagulable state, the recommendation is to keep women who are at higher risk -such as those who are not regularly transfused and those splenectomised- on prophylaxis during pregnancy and the postpartum period.

Keywords: Counselling; Hemoglobin H disease; Pregnancy; Thalassemia intermedia; Thalassemia major.

Figure 1

Schematic representation of the fertility options for a woman with thalassemia wishing to become pregnant. Abbreviations: IVF, In vitro fertilization. Please note that choices concerning fertility are strictly personal and involve ethical, emotional, cultural, financial and practical aspects.