In Haemoglobin H disease, acute haemolytic crisis is a recognised dominant clinical feature. It presents as acute worsening of anaemia, frequently accompanied by jaundice and/or dark-coloured urine, requiring prompt assessment and intervention.
The primary objective in this acute setting is to restore haemoglobin to 80–90 g/L.
Management centres on red cell transfusion together with adequate intravenous hydration. Additional targeted measures are applied according to clinical findings present at the time of the episode.
A haemolytic crisis is considered a dominant clinical feature of α-thalassaemia, and it is characterized by acute worsening of anaemia, frequently in combination with jaundice and/or dark-coloured urine.
During an acute haemolytic episode, blood transfusion should be provided to restore Hb to 80–90 g/L, along with adequate intravenous hydration.