The interaction of HbE with ß-thalassaemia results in HbEß-
thalassaemia, an extremely heterogeneous clinical
condition. HbE-ß-thalassaemia is the most common form
of ß-thalassaemia in Southeast Asia and accounts for
approximately 50% of cases of transfusion –dependent
cases of haemolytic anaemia. Still an undiagnosed
population exist in our midst often presenting with
complications of chronic haemolysis along with anaemia.
Obstetricians are further perplexed while managing these
patients in pregnancy with added foetal risks. Pulmonary
hypertension, cardiac arrhythmia, systemic iron overload
from chronic blood transfusion usually evolves from the
disease itself.The risk of the foetus inheriting the trait of
either ²-thalassemia or Hb-E exists(25% in each pregnancy)
along the possibility of being homozygous for this disorder
if the father bears the carrier status(25 % in each
pregnancy) cannot be overlooked. Here we report a 20-
year old primigravida with Hb E- ß thalassemia presenting
at 40 weeks of pregnancy with severe anaemia (4 gm/dl)
and respiratory distress. The patient also had
hepatosplenomegaly and cholelithiasis. The patient had
remained undiagnosed upto the time of presentation and
had remarkably received no blood transfusion since
childhood despite the history of recurrent jaundice. The
patient was further investigated and found to have
moderate pulmonary hypertension and mild tricuspid
regurgitation. After correction of her anaemia and
supportive cardiac care, she delivered a male child of
2.75kg by caesarean section. Her cardiac condition also
significantly improved after delivery.
Key words: Thalassemia, Hb E-â thalassemia, pregnancy,
pulmonary hypertension.
(J Bangladesh Coll Phys Surg 2019; 37: 43-45)