Pregnancy and  -thalassemia: an Italian multicenter experience

Abstract

BackgroundRecent advances in the management of thalassemia have significantly improved life expectancy and quality of life of patients with this hemoglobinopathy, with a consequent increase in their reproductive potential and desire to have children. Design and MethodsWe describe the methods of conception and delivery, as well as the course and outcome of pregnancy including transfusions, iron overload and chelation in 46 women with thalassemia major (58 pregnancies) and in 11 women with thalassemia intermedia (17 pregnancies). Conception was achieved after gonadotrophin-induced ovulation in 33 of the women with thalassemia major and spontaneously in all of those with thalassemia intermedia. ResultsAmong the women with thalassemia major, 91% of the pregnancies resulted in successful delivery of 45 singleton live-born neonates, five sets of twins and one set of triplets. No secondary complications of iron overload developed or worsened during pregnancy. When considering only the singleton pregnancies, the proportion of babies with intrauterine growth retardation did not differ from that reported in the general Italian population. The high prevalence of pre-term births (32.7%) was mostly related to multiple pregnancies and precautionary reasons. Pregnancy was safe in most women with thalassemia major or intermedia. However, women with thalassemia intermedia who had never previously been transfused or who had received only minimal transfusion therapy were at risk of severe alloimmune anemia if blood transfusions were required during pregnancy. ConclusionsProvided that a multidisciplinary team is available, pregnancy is possible, safe and usually has a favorable outcome in patients with thalassemia. In women with hypogonadotropic hypogonadism, gonadal function is usually intact and fertility is usually retrievable.Key words: pregnancy, thalassemia major, thalassemia intermedia, hypogonadism, assisted reproduction.Citation: Origa R, Piga A, Quarta G, Forni GL, Longo F, Melpignano A, and Galanello R. Pregnancy and β-thalassemia: an Italian multicenter experience. Haematologica. 2010; 95:376-381. doi:10.3324/haematol.2009 This is an open-access paper.Pregnancy and β β-thalassemia: an Italian multicenter experience

Reproductive capacity in iron overloaded women with thalassemia major

“…Indeed, case series of successful pregnancy induction report a mean age of 24.5 and 29.5 years. 2,13 The rate of decline in ovarian reserve varies considerably between individual women and is probably even more variable among women with TM. Therefore, a test that can provide information regarding the status of a woman's remaining oocytes is of great importance.…”

The life of patients with thalassemia major

“…15 Overall, hundreds of pregnancies have taken place in women with thalassemia, but pre-existing cardiomyopathy, expanded plasma volume, increased cardiac output, and reduced glucose tolerance all represent menaces to maternal health. The study by Origa et al, published in this issue of the journal, 16 is an important testimony on how improvements in therapy have completely changed the long-term prognosis and quality of life of thalassemic patients. The authors report on the course of 75 pregnancies in 46 women with thalassemia major and in 11 women with thalassemia intermedia followed in four Italian centers participating in a cooperative study.…”

Cardiovascular Function and Treatment in β-Thalassemia Major

“…287 Iron overload may result in cardiac complications, and therefore, we recommend assessment of heart T2* and cardiac function and dimensions before conception. Blood consumption may increase during pregnancy to maintain a hemoglobin level of ≈10 g/dL and ensure optimal fetal growth, 283 and when combined with the interruption of chelation because of teratogenic considerations, this may significantly worsen iron overload. These factors, the increased blood volume and changes in blood pressure, may compromise heart function, which should be monitored carefully during pregnancy.…”

“…Unfortunately, the majority have impaired fertility caused by hypogonadism and require induction of ovulation, which may produce a high number of twin or triplet pregnancies. [283][284][285][286] The maternal and fetal risks depend primarily on preexisting maternal complications and iron-related organ damage. 287 Iron overload may result in cardiac complications, and therefore, we recommend assessment of heart T2* and cardiac function and dimensions before conception.…”

“…An increasing number of woman with TM have a successful pregnancy, with >500 pregnancies reported. 22,[280][281][282][283] Spontaneous fertility can occur in well-transfused and chelated women with TM. Unfortunately, the majority have impaired fertility caused by hypogonadism and require induction of ovulation, which may produce a high number of twin or triplet pregnancies.…”