ORIGINAL ARTICLE
Pregnancy in beta-thalassemia intermedia: 20-year
experience of a Greek thalassemia center
Ersi Voskaridou
1
, Angeliki Balassopoulou
1
, Effrossyni Boutou
1
, Veroniki Komninaka
1
, Dimitrios
Christoulas
2
, Maria Dimopoulou
1
, Evangelia-Eleni Delaki
1
, Dimitris Loukopoulos
3
, Evangelos Terpos
4
1
Thalassemia Centre, ‘Laiko’ University General Hospital of Athens, Athens;
2
Department of Hematology, 251 General Air Force Hospital, Athens;
3
Hematology Research Laboratory, Biomedical Research Foundation, Academy of Athens, Athens;
4
Department of Clinical Therapeutics, School
of Medicine, National and Kapodistrian University of Athens, Athens, Greece
Abstract
Objective: Progress in the management of patients with thalassemia intermedia (TI) enabled increasing
rates of pregnancies among TI women worldwide. Nevertheless, information regarding TI pregnancy
management and outcome is quite limited in the literature. The aim of this study was to report our
experience regarding the maternal and fetal outcome of TI patients, as well as to depict the complexity of
the disease and the need for multidisciplinary and personalized management as shown by the description
of two interesting pregnancy cases. Methods: We analyzed our data recorded from 60 pregnancies in 34
women over a 20-yr period. Results: Forty-nine patients achieved full-term pregnancies (mean maternal
age SD: 27.4 6.5 yr) within 37 3 gestation weeks. Their mean hemoglobin value was
8.33 1.22 g/dL; 26.5% of patients were not transfused at all or they had been transfused only once
during gestation. There were 11 abortions (18.3%). The spontaneous abortions (5/11) were related to high
HbF levels. Six patients had more than two normal deliveries. Nineteen newborns (38.8%), which weighed
2–3 kg, required hospitalization to an intensive neonatal care unit for 1–3 wk. One patient presented with
life-threatening complications (hemolytic anemia, thrombocytopenia, and enlargement of spleen) and
another with spastic paraparesis due to extramedullary paravertebral masses. Conclusions: Although
several complications can occur during a pregnancy in TI women, the careful and frequent monitoring by
both hamatologists and obstetricians can lead to successful deliveries.
Key words pregnancy; thalassemia intermedia; transfusion
Correspondence Ersi Voskaridou, Thalassemia Center, Laiko University General Hospital, 16 Sevastoupoleos Street, GR 11526
Athens, Greece. Tel: +302132060940; Fax: +302107456138; e-mail: ersi_voskaridou@yahoo.com
Accepted for publication 27 May 2014 doi:10.1111/ejh.12387
Thalassemia is the most commonly inherited disease
worldwide. Thalassemia intermedia (TI) represents a phe-
notype ranging from thalassemia major (TM) to thalasse-
mia minor. Fetal hemoglobin (HbF), hemoglobin A, or a
combination of both are produced in TI patients in vary-
ing levels according to the underlying beta locus mutations
(1). TI patients have with a milder clinical appearance
than those with TM that varies from being completely
asymptomatic and transfusion independent until adulthood,
to requiring regular transfusions from early childhood
(>2 yr old) with severe growth retardation and skeletal
deformities (2). There is no adequate clinical definition of
TI; it is a diagnosis that can be made only after a consid-
erable period of observation and that often requires revi-
sion. Individuals with thalassemia intermedia present later
than thalassemia major, have milder anemia, and by
definition do not require or only occasionally require
transfusion. In contrast, the clinical presentation of thalas-
semia major occurs between 6 and 24 months and requires
regular red blood cell (RBC) transfusions to survive.
The majority of patients with TI present with mild anemia
(Hb: 7–10 g/dL) and splenomegaly, symptoms that are
492 © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd
European Journal of Haematology 93 (492–499)