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May 30, 1977

Ravages of Copper in Early Wilson's Disease

JAMA. 1977;237(22):2413. doi:10.1001/jama.1977.03270490053030

Transient intravascular hemolysis as an isolated presentation of Wilson's disease was first reported in 1934.1 Sporadic reports of this phenomenon, which may precede other symptoms of hepatolenticular degeneration by several years, have appeared since. In this issue, Robitaille and associates (p 2402) describe three additional cases of Wilson's disease with transient hemolytic anemia as a presenting manifestation, which was associated with an unexpected and unexplained elevation of A2 hemoglobin. The transient hemolysis was not an isolated manifestation. One patient had an enlarged liver; another had ascites and cholestatic jaundice. The involvement of the liver did not, however, substantially affect prognosis. All three patients responded to penicillamine therapy.

A happy ending does not, unfortunately, follow every case of intravascular hemolysis associated with hepatic involvement. Roche-Sicot and Benhamou2 report three young patients with Wilson's disease in whom this association resulted in a fatal outcome. Death was due not to