This is a common cause of fetal loss throughout South-East Asia and is also encountered in the Mediterranean region. There is no production of a-chains and hence neither fetal nor adult haemoglobin. The fetus is usually stillborn between 28 and 40 weeks, or, if liveborn, takes a few gasping respirations and then expires within the first hour after birth. Affected neonates show the typical picture of hydrops fetalis, with gross pallor, generalized oedema and massive hepatosplenomegaly. There is an increased frequency of congenital abnormalities and a very large friable placenta. All these findings are due to severe intrauterine anaemia. The haemoglobin is in the 6-8 g/dL range and there are gross thalassaemic changes of the red cells, with many nucleated forms in the blood. The haemoglobin consists of approximately 80% Hb Bart's and 20% Hb Portland (Z2Y2). It is believed that these infants survive to term because they continue to produce embryonic haemoglobin. Apart from fetal death, this syndrome is characterized by a high incidence of toxaemia of pregnancy and obstetric complications due to the large placenta.
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