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非输血依赖型地中海贫血(NTDT)女性的妊娠结局:血液学中心经验

Pregnancy outcomes in women with non-transfusion dependent thalassaemia (NTDT): A haematology centre experience.

作者信息

Lee B S, Sathar J, Sivapatham L, Lee L I

机构信息

Ampang Hospital, Department of Haematology, Malaysia.

出版信息

Malays J Pathol. 2018 Aug;40(2):149-152.

Abstract

INTRODUCTION

Non-transfusion dependent thalassaemia (NTDT) is a term used for thalassaemia patients who do not require lifelong regular transfusions for survival. Pregnancy in these women, whether spontaneous or through assisted reproductive technology, represents a challenge for the physician.

MATERIALS AND METHODS

The maternal and foetal outcomes of patients with NTDT followed up in a tertiary haematology centre over 6 months period were studied. A total of 36 pregnancies in 26 pregnant women with NTDT were analysed.

RESULTS

Among these women, all of the pregnancies resulted in successful delivery of singleton live-born neonates. There were four clinically distinct forms of NTDT among these women which include Hb E/β-thalassemia (mild and moderate forms), HbH disease, HbH-Constant Spring, and homozygous δβ-thalassemia. No blood transfusion was needed in 15 of the 36 pregnancies (41.6%). The lowest mean Hb level in which no blood transfusion was given was 8.21 g/dL. The mean of packed-cell units received during pregnancy was 6.95 units per pregnancy. There was no worsening of serum ferritin observed during pregnancy with mean serum ferritin pre- and post-pregnancy of 409.35 ug/L and 418.18 ug/L respectively. The mean gestational age at delivery was 38.6 weeks with no preterm delivery reported. The mean foetal birth weight was 2729 grams. There was no intrauterine growth restriction (IUGR) or congenital malformation. There was a case of small for gestational age (SGA) and a case of oligohydramnios.

CONCLUSION

This study showed that pregnancy was possible, safe and has a favourable outcome in patients with NTDT with multidisciplinary care.

摘要

引言

非输血依赖型地中海贫血(NTDT)是用于描述那些生存不需要终身定期输血的地中海贫血患者的术语。这些女性怀孕,无论是自然受孕还是通过辅助生殖技术受孕,对医生来说都是一项挑战。

材料与方法

对在一家三级血液学中心随访6个月的NTDT患者的母婴结局进行了研究。分析了26名患有NTDT的孕妇的36次妊娠情况。

结果

在这些女性中,所有妊娠均成功分娩出单胎活产新生儿。这些女性中有四种临床上不同类型的NTDT,包括Hb E/β地中海贫血(轻度和中度形式)、HbH病、HbH-Constant Spring和纯合子δβ地中海贫血。36次妊娠中有15次(41.6%)不需要输血。未输血的最低平均血红蛋白水平为8.21 g/dL。孕期接受的红细胞压积单位平均每次妊娠为6.95单位。孕期未观察到血清铁蛋白恶化,妊娠前和妊娠后的平均血清铁蛋白分别为409.35 ug/L和418.18 ug/L。分娩时的平均孕周为38.6周,无早产报告。胎儿平均出生体重为2729克。无宫内生长受限(IUGR)或先天性畸形。有1例小于胎龄儿(SGA)和1例羊水过少病例。

结论

本研究表明,通过多学科护理,NTDT患者怀孕是可能的、安全的且有良好结局。

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