The DOI matches its Crossref record; 21 of the 21 references it cites were found in a registry or an index.
The Interaction of Alpha-Thalassemia and Homozygous Sickle-Cell Disease
Abstract
Patients with homozygous sickle-cell disease may be homozygous for alpha-thalassemia 2 (alpha-/alpha-), may be heterozygous for alpha-thalassemia 2 (alpha-/alpha alpha), or may have a normal alpha-globin-gene complement (alpha alpha/alpha alpha). We compared the clinical and hematologic features of 44 patients who had sickle-cell disease and homozygous alpha-thalassemia 2 with those of controls with the two hematologic conditions. The patients with homozygous alpha-thalassemia 2 had significantly higher red-cell counts and levels of hemoglobin and hemoglobin A2, as well as significantly lower hemoglobin F, mean corpuscular hemoglobin, mean corpuscular hemoglobin concentration, mean corpuscular volume, reticulocyte counts, irreversibly-sickled cell counts, and serum total bilirubin levels, than those with a normal alpha-globin-gene complement. Heterozygotes (alpha-/alpha alpha) had intermediate values. In the group with homozygous alpha-thalassemia 2, fewer patients had episodes of acute chest syndrome and chronic leg ulceration and more patients had splenomegaly, as compared with patients in other two subgroups. These data confirm previous suggestions that alpha-thalassemia inhibits in vivo sickling in homozygous sickle-cell disease and may be an important genetic determinant of its hematologic severity.
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Citations
Cites
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Concurrent Sickle-Cell Anemia and α-Thalassemia: Effect on Severity of Anemia
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Self-citationIndividual variation in the production and survival of F cells in sickle-cell disease
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Cited by
Rees, D. C., Williams, T. N., & Gladwin, M. T. (2010) · The Lancet · doi:10.1016/s0140-6736(10)61029-x · Cited by 2,504
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Fetal hemoglobin in sickle cell anemia: determinants of response to hydroxyurea
Steinberg, M. H., Lu, Z.-H., Barton, F. B., Terrin, M. L., Charache, S., Dover, G. J., & Hydroxyurea, T. M. S. O. (1997) · Blood · doi:10.1182/blood.v89.3.1078 · Cited by 407
The spleen and sickle cell disease: the sick(led) spleen
Brousse, V., Buffet, P., & Rees, D. (2014) · British Journal of Haematology · doi:10.1111/bjh.12950 · Cited by 294
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Metadata
- Authors
- Higgs, D. R., Aldridge, B. E., Lamb, J., Clegg, J. B., Weatherall, D. J., Hayes, R. J., Grandison, Y., Lowrie, Y., Mason, K. P., Serjeant, B. E., & Serjeant, G. R.
- Venue
- New England Journal of Medicine
- Publisher
- Massachusetts Medical Society
- Published
- 1982-06-17
- Volume
- 306
- Issue
- 24
- Pages
- 1441-1446
- ISSN
- 0028-4793 (ISSN-L)
- 1533-4406
- OpenAlex
- W2066521171
- Cited by
- 313
- References
- 21
- FWCI
- 9.92
- Type
- journal article
- Language
- en
- Open access
- closed
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- Higgs, D. R., Aldridge, B. E., Lamb, J., Clegg, J. B., Weatherall, D. J., Hayes, R. J., Grandison, Y., Lowrie, Y., Mason, K. P., Serjeant, B. E., & Serjeant, G. R. (1982). The Interaction of Alpha-Thalassemia and Homozygous Sickle-Cell Disease. New England Journal of Medicine. https://doi.org/10.1056/nejm198206173062402
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