From perpetual haemosiderinuria to possible iron overload: iron redistribution in paroxysmal nocturnal haemoglobinuria patients on eculizumab by magnetic resonance imaging.

We investigated iron compartmentalization in 20 haemolytic PNH patients. Standard biochemical testing, including iron parameters and flow cytometry for complement component 3 (C3) on erythrocytes, was combined with magnetic resonance imaging (MRI) to assess calculated iron content (CIC) of kidneys, liver and spleen. Three distinct patterns of iron tissue deposition were identified in the PNH patients receiving eculizumab: (i) Low iron levels in liver, spleen and kidneys; (ii) high iron levels in liver and/or spleen and low in kidneys; (iii) high iron levels in liver, spleen and kidney. The possible long-term consequences of iron redistribution during eculizumab treatment and the possible need of specific therapeutic interventions will have to be assessed in future clinical studies.