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 life of patients with thalassemia, 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 during preconception usually carry out a successful gestation and labour, both in case of spontaneous conception and use of assisted reproductive techniques. A multidisciplinary team including a cardiologist, an endocrinologist and a gynaecologist, with 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. Haemoglobin should be maintained over 10 g/dL to to allow normal foetal growth. Chelators are not recommended during pregnancy; nevertheless, it may reasonable to consider restarting chelation therapy with desferrioxamine towards the end of the second trimester when the potential benefit outweighs the potential foetal 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 anaemia 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 during the postpartum period.   Keywords:Pregnancy, Thalassemia major, Thalassemia intermedia, Haemoglobin H disease, Counselling