Outcome of Thalassemia Intermedia Mothers and Newborns in Two Tertiary Care Centers, Beirut and Milan.

Introduction: β-thalassemia intermedia (TI) patients either present with a severe disease between the ages of 2–6 years, or remain asymptomatic until they present with a milder disease during adult life. With the advances in assisted reproductive technology and medical management, pregnancy has become possible in most TI cases. Spontaneous abortions, intrauterine growth restriction (IUGR), and preterm labor are complications that face women with TI. This study aimed at evaluating the pregnancy outcome of women with TI in two tertiary care centers, one in Lebanon and another in Italy.

Methods: Patient databases at the Chronic Care Center, Lebanon and at the Hereditary Anemia Center in Milan, Italy were reviewed. Maternal and neonatal charts of women with TI and documented pregnancy were identified and reviewed using a specially formulated questionnaire that focused on demographic characteristics (maternal age, gestational age (GA), parity, and abortions), past medical history related to thalassemia, course of pregnancy and complications, intrapartum events and neonatal outcome. Patients were then contacted by phone for any missing information.

Results: A total of 44 pregnant TI patients, 11 in Lebanon and 33 in Italy were identified. Mean age at diagnosis of TI was 9.2 ± 9.7 years (range 1–42 years) with 34 (77.3%) splenectomized patients identified. Fourteen patients (31.8%) were frequently transfused, 25 (56.8%) occasionally transfused and only 4 (9.1%) never transfused. Mean age at pregnancy was 29.5 ± 3.9 years (range 21–38 years) with 11 patients (28.2%) receiving the first transfusion during pregnancy. Twenty eight patients were on chelation therapy: 21 (48.8%) received deferioxamine, 4 (9.3%) deferiprone and 3 (7%) deferasirox. After excluding 4 women with ongoing pregnancies, information on 79 pregnancies, all spontaneous, were available, 30 from Lebanon and 49 from Italy. These resulted in 60 live births (75.9%), 17 abortions (21.5%), and 2 intrauterine fetal deaths (2.5%), at 26 and 36 weeks of gestation, respectively. Three patients (7.5%) had recurrent abortions (≥3 abortions). Of women whose pregnancies progressed beyond 20 weeks of gestation (n=62), the following obstetrical complications were encountered: preterm delivery <37 weeks of gestation (18, 29.0%), IUGR (14, 22.6%), preeclampsia (1, 1.6%), placental abruption (2, 3.2%) and cholestasis of pregnancy (1, 1.6%). Mean GA at delivery was 36.8 ±2.8 weeks. Thrombotic events were encountered during 4/79 (5.1%) pregnancies: deep vein thrombosis (DVT) ante- and post-partum (n=2), antepartum DVT (n=1) and placental thrombosis (n=1). Two women had postpartum splenectomy. Cesarean delivery was performed in 44 pregnancies (70.9%). No significant differences were noted when the course of pregnancy and obstetrical outcomes of women in Lebanon and Milan were compared.

Conclusions: Preterm delivery complicates 29% of pregnancies with TI which are at a 23% risk of IUGR. Cesarean delivery is the most common route of delivery in these patients (70.9%). As with any chronic disease affecting different organs, TI presents a challenge for pregnant patients, their fetuses and physicians and requires close monitoring for any events that might compromise the patient or the newborn. Further studies comprising more patients need to be carried out to determine the effects of splenectomy and transfusions during pregnancy and on the outcome of those pregnancies.