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

Pregnancy in Thalassemia and Sickle Cell Disease: The Experience of an Italian Thalassemia Center

“…As regards thalassemia patients, despite the physiologic decline in fertility, and follicle aging depending on the iron toxicity, the ovarian function is preserved in transfusion-dependent thalassemia. Data on the frequency of failure in ovulation induction or timeline to a successful pregnancy are limited (Ansari et al, 2006;Voskaridou et al, 2014;Origa and Comitini, 2019). Crucial points in the management of pregnancies are the iron chelation therapy before and during pregnancy, the antithrombotic prophylaxis, the management of transfusion therapy according to the modified transfusion requirement, paying particular attention to immune-hematological examinations, and a careful cardiologic monitoring for hemodynamic changes that expose an increased risk of heart failure.…”

“…In addition, non-transfusion dependent thalassemia intermedia patients can need transfusion for the first time in pregnancy due to the dilution of the hemoglobin. In some cases, it may be appropriate to reduce the transfusion interval and to administer only one unit of red blood cells at a time to support the fetal oxygenation requirements (Origa and Comitini, 2019). Two cases of SCD patients (HbS/HbC) needed the transfusion of one unit of red blood cells during pregnancy for vaso-occlusive crisis.…”

“…The increase in life expectancy has consequently changed patients' perspectives, leading them to longer-term planning with a consequent increase in their reproductive potential and desire to have children. Hypogonadism still represents an obstacle for some patients, but the progress in assisted reproduction techniques has helped many of them to fulfill this wish (Leung and Lao, 2012;Lao, 2017;Materazzi et al, 2017a;Carlberg et al, 2018;Origa and Comitini, 2019).…”

The History of Deferiprone (L1) and the Complete Treatment of Iron Overload in Thalassaemia

“…118,119 Changes in the therapeutic strategies are necessary under special circumstances such as pregnancy, splenomegaly, and infections and also when considering the possible introduction of erythropoietic biological or other emerging therapies. [120][121][122][123] Similar considerations are in progress regarding other clinical issues such as the early initiation of chelation therapy using L1 in thalassaemia children from about one year of age and also the initiation of combination therapies. [124][125][126][127] There are different criteria and opinions regarding the latter, with the ICOC L1/DF combination, for example, to be available, safe and flexible in all the patient categories and cases depending on the iron load levels and the rate of body iron intake from transfusions, whereas for other groups of investigators different restrictions are imposed in the use of combination protocols (Figure 6).…”

Current Perspective of Beta Thalassemia and Its Treatment Strategies

“…Beta-thalassemia might be prevented by identification of carrier, prenatal diagnosis and genetic counseling [37]. Genetic counseling offers evidence for risk of carriers of both parents and risk in offspring.…”