Clinical Pharmacology and Therapeutics
Clinical Pharmacology and Therapeutics
5 years ago

Haemolysis during sodium dimercaptosulphonate therapy for Wilson's disease in G6PD-deficient patients: First report of two cases

Haemolysis during sodium dimercaptosulphonate therapy for Wilson's disease in G6PD-deficient patients: First report of two cases
Y. Q. Huang, H. W. Wu, A. Q. Liu, S. Lai
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. The bone marrow was taken from spina iliaca posterior superior and was Wright staining (×1000). From the bone marrow aspirate smear, abnormal cells were not observed. The shape of mature red blood cells, platelet, erythroblast were normal. But the proportion of erythroblast had increased, accounting for 45%. And extracellular iron is positive (2+) and the proportion of intracellular iron is 50%. The bone marrow aspirate smear reveals that the bone marrow and the red blood cell was active proliferation, known as erythrocytosis.

Publisher URL: http://onlinelibrary.wiley.com/resolve/doi

DOI: 10.1111/jcpt.12576

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