Department of Obstetrics and Gynecology, American University of Beirut Medical Center, Beirut, Lebanon
2
Chronic Care Center, Beirut, Lebanon
3
Department of Internal Medicine, American University of Beirut Medical Center, Beirut, Lebanon
Little is known about the outcome of pregnancy in women with b-thalassemia intermedia
(TI). Over 10 years, maternal and neonatal outcomes of women with TI followed at a single
thalassemia center were reviewed. Nine spontaneous pregnancies in ve women with TI
were studied. Six pregnancies resulted in live newborns; two were complicated by rsttrimester abortions and one by an unexplained intrauterine fetal death at 36 weeks gestation. Two patients had splenectomy before pregnancy: one required cesarean delivery and
splenectomy at 312/7 weeks gestation for worsening hemolytic anemia and thrombocytopenia and another had splenectomy 8 weeks postpartum for symptomatic hypersplenism. Two
patients had received transfusions before pregnancy, and two required them for the rst
time during pregnancy and developed antibodies, which contributed to worsening of their
anemia and repeated transfusions. The mean number of transfusions received during pregnancy was 8.0 5.2 units. The mean lowest hemoglobin level in pregnancy was 5.2 2.0 g/dl.
Cesarean delivery was performed in 42.9% of cases. Mean gestational age at delivery was
36.7 3.1 weeks with intrauterine growth restriction (IUGR) complicating 57.1% of cases. In
conclusion, IUGR complicates more than half of pregnancies with TI. Transfusions are needed
in most cases, even in non-transfusion-dependent patients. Postpartum splenectomy might be
C 2006 Wiley-Liss, Inc.
necessary in some patients. Am. J. Hematol. 81:499502, 2006. V
Key words: b-thalassemia intermedia; obstetric complications; neonatal outcome
INTRODUCTION
Transfusion-independent thalassemic patients suffering from anemia with hemoglobin levels (Hb) of
710 g/dl and variable degrees of splenomegaly are
classied as b-thalassemia intermedia (TI) [1,2].
Patients with TI have, in general, a milder clinical
phenotype than those with thalassemia major. At
the severe end of the clinical spectrum of TI,
patients are usually diagnosed between the ages of 2
and 6 years old. Although they survive without regular transfusions, growth and development are retarded. At the other end of the spectrum, there are
patients who are completely asymptomatic until
adulthood, when they present with anemia and splenomegaly often found by chance during hematological examinations or family studies [1,2].
Although fertility is compromised in patients with
transfusion-dependent thalassemia major, pregnancy
is possible in the majority of patients with TI. This is
particularly true with the availability of assisted
C 2006 Wiley-Liss, Inc.
V
Presented at the 25th Annual Meeting of the Society for Maternal Fetal Medicine in Reno, Nevada, Feb 712, 2005.
*Correspondence to: Anwar H. Nassar, Department of Obstetrics and Gynecology, American University of Beirut Medical
Center, P.O. Box 113-6044/B36, Beirut, Lebanon.
E-mail: an21@aub.edu.lb
Received for publication 2 August 2005; Accepted 18 December
2005
Published online in Wiley InterScience (www.interscience.wiley.com).
DOI: 10.1002/ajh.20654
500
Nassar et al.
spontaneous abortions, preterm labor, and intrauterine growth restriction (IUGR) [3,4]. In Lebanon,
approximately one third of thalassemia patients have
TI [5]. A study that analyzed the molecular basis of
TI has shown that the most contributing factor in
this population is the b-genotype where 68% of
patients have a mild b mutation (IVSI-6, cd29,
88, or 87), while 26% are positive for the XmnI
polymorphism associated with increased production
of fetal hemoglobin (HbF), which shows strong linkage to certain mutations (IVSII-1, cd8, and cd30) [5].
The purpose of this study is to review the maternal
and fetal outcome of patients with TI followed up in
a single center with standard protocols.
MATERIALS AND METHODS
Several small studies and case series have demonstrated favorable obstetrical and fetal outcome in
patients with b-thalassemia major when close followup and intensive treatment are instituted [69]. Less is
known about pregnancy performance in patients with
TI [6,8]. This entity encompasses a wide clinical spectrum. These patients usually have a disease the
severity of which is somewhere between the mild manifestations of thalassemia trait and the severe symptoms of thalassemia major. Our series, one of the larg-
501
Patient
Mutation
Hb F
(%)
No. of
preg.
First
transfusion
Splenectomy
IUGR
Outcome
Cesarean delivery, full term
Cesarean delivery at 31 weeks
1st preg.: Cesarean delivery, full term
2nd preg.: 1st-trimester abortion
1st preg.: vaginal delivery, full term
2nd preg.: vaginal delivery, full term
3rd preg.: vaginal delivery at 36 weeks
1st preg.: 1st-trimester abortion
2nd preg.: IUFD at 36 weeks, vaginal delivery
1
2
3
IVSI-5/IVSI-5
IVSI-6/IVSI-6
IVSI-6/IVSI-6
80.1
94.8
60.0
1
1
2
None
Index preg.
5 years
Intact
Postpartum
Prior to pregnancy
cd29/cd29
17.1
2nd preg.
IVSI-110/IVSI-6
17.4
13 years
Prior to pregnancy
Note: Preg., pregnancy; IUGR, intrauterine growth restriction; IUFD, intrauterine fetal death.
502
Nassar et al.
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