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Hemolytic Anemia in Wilson's Disease
Abstract
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.
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Citations
Cited by
Essentiality of copper in humans
Uauy, R., Olivares, M., & Gonzalez, M. (1998) · The American Journal of Clinical Nutrition · doi:10.1093/ajcn/67.5.952s · Cited by 1,052
Liver cell death and anemia in Wilson disease involve acid sphingomyelinase and ceramide
Lang, P. A., Schenck, M., Nicolay, J. P., Becker, J. U., Kempe, D. S., Lupescu, A., Koka, S., Eisele, K., Klarl, B. A., Rübben, H., et al. (2007) · Nature Medicine · doi:10.1038/nm1539 · Cited by 438
The role of ceruloplasmin in iron metabolism.
Roeser, H. P., Lee, G. R., Nacht, S., & Cartwright, G. E. (1970) · Journal of Clinical Investigation · doi:10.1172/jci106460 · Cited by 365
Oral zinc therapy for Wilson's disease
BREWER, G. J., HILL, G. M., PRASAD, A. S., COSSACK, Z. T., & RABBANI, P. (1983) · Annals of Internal Medicine · doi:10.7326/0003-4819-99-3-314 · Cited by 244
Oxidative stress, eryptosis and anemia: a pivotal mechanistic nexus in systemic diseases
Bissinger, R., Bhuyan, A. A. M., Qadri, S. M., & Lang, F. (2019) · The FEBS Journal · doi:10.1111/febs.14606 · Cited by 239
Metadata
- Authors
- DEISS, A., LEE, G. R., & CARTWRIGHT, G. E.
- Venue
- Annals of Internal Medicine
- Publisher
- American College of Physicians
- Published
- 1970-09-01
- Volume
- 73
- Issue
- 3
- Pages
- 413-418
- ISSN
- 0003-4819 (ISSN-L)
- 1539-3704
- OpenAlex
- W2091660130
- Cited by
- 162
- References
- 0
- FWCI
- 10.03
- Type
- journal article
- Language
- en
- Open access
- closed
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- DEISS, A., LEE, G. R., & CARTWRIGHT, G. E. (1970). Hemolytic Anemia in Wilson's Disease. Annals of Internal Medicine. https://doi.org/10.7326/0003-4819-73-3-413
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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