investigations

Last reviewed 09/2022

• the FBC shows anaemia of variable severity (Hb 5-10 g/l)
• the oxygen dissociation curve is displaced to the right as a result of the raised intracellular 2,3-diphosph- glycerate
• the blood film shows prickle cells and reticulocytosis
• autohaemolysis is increased and glucose is not protective in contrast to hereditary spherocytosis
• pyruvate kinase is only 5-20% of normal levels