A. DEISS, M.D.; G. R. LEE, M.D., F.A.C.P.; G. E. CARTWRIGHT, M.D., F.A.C.P.
Penicillamine therapy was discontinued for a period of 200 days in a patient with Wilson's disease. Copper reaccumulated in the body at the rate of about 0.43 mg/day. Urinary copper remained normal for 180 days and then increased rapidly. Simultaneously with the onset of hypercupriuria serum copper, ceruloplasmin, and erythrocyte copper increased, and hemolytic anemia associated with the formation of Heinz bodies on exposure to acetylphenylhydrazine developed. Addition of copper to normal erythrocytes led to increased autohemolysis, thermolability of hemoglobin, increased sensitivity to acetylphenylhydrazine, decreased erythrocyte glutathione, decreased glutathione stability, and inhibition of glucose-6-phosphate dehydrogenase and glutathione reductase. The hemolytic anemia was due to increased oxidative stress on the erythrocytes as a consequence of the accumulation of copper within the cells.
DEISS A, LEE GR, CARTWRIGHT GE. Hemolytic Anemia in Wilson's Disease. Ann Intern Med. ;73:413–418. doi: 10.7326/0003-4819-73-3-413
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Published: Ann Intern Med. 1970;73(3):413-418.
Encephalopathy, Gastroenterology/Hepatology, Hematology/Oncology, Liver Disease, Neurology.
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