What is known and objective: Wilson's disease (WD) is an inherited disorder in which defective biliary excretion of copper leads to its accumulation. Sodium dimercaptosulphonate (DMPS) is used as the primary therapy in China.
Case description: We report two cases, with WD and G6PD deficiency, who developed haemolysis on treatment with DMPS, without any other known risk. After withdrawal of DMPS and administration of dexamethasone and packed red blood cells, the patients recovered.
What is new and conclusion: Clinicians should keep in mind haemolysis as a potentially life-threatening side effect of DMPS in patients with G6PD.
Keywords: Wilson's disease; adverse effect; glucose-6-phosphate; haemolysis.
© 2017 John Wiley & Sons Ltd.