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Alpha thalassemia changes erythrocyte heterogeneity in sickle cell disease.
J Clin Invest. 1985 May; 75(5):1632-7.JCI

Abstract

Homozygous alpha-thalassemia has the beneficial effect in sickle cell anemia of reducing the hemolytic severity while changing several other hematological parameters. We examined in detail the cellular basis of some of these hematologic alterations. We find that the broad distribution in erythrocyte density and the large proportion of dense cells associated with sickle cell anemia are both reduced with coexisting alpha-thalassemia. Measurements of glycosylated hemoglobin levels as a function of cell density indicate that the accelerated increase in cell density, beyond normal cell aging, in sickle cell anemia is also reduced with alpha-thalassemia. The patients with homozygous alpha-thalassemia and sickle cell disease have slightly lower levels of hemoglobin F than the nonthalassemic sickle cell patients. Examination of hemoglobin F production revealed that the proportion of hemoglobin F containing reticulocytes remained unchanged, as did the proportion of hemoglobin F in cells containing hemoglobin F (F cells). Preferential survival of F cells occurs in sickle cell anemia, with or without alpha-thalassemia, and the slight difference in hemoglobin F levels appear to reflect differences in numbers of circulating F cells. Thus, in sickle cell disease with coexisting alpha-thalassemia, the change in the erythrocyte density profile, possibly due to inhibition of polymerization-related increases in cell density, explains the hematological improvement.

Authors

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Pub Type(s)

Comparative Study
Journal Article
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

2581999

Citation

Noguchi, C T., et al. "Alpha Thalassemia Changes Erythrocyte Heterogeneity in Sickle Cell Disease." The Journal of Clinical Investigation, vol. 75, no. 5, 1985, pp. 1632-7.
Noguchi CT, Dover GJ, Rodgers GP, et al. Alpha thalassemia changes erythrocyte heterogeneity in sickle cell disease. J Clin Invest. 1985;75(5):1632-7.
Noguchi, C. T., Dover, G. J., Rodgers, G. P., Serjeant, G. R., Antonarakis, S. E., Anagnou, N. P., Higgs, D. R., Weatherall, D. J., & Schechter, A. N. (1985). Alpha thalassemia changes erythrocyte heterogeneity in sickle cell disease. The Journal of Clinical Investigation, 75(5), 1632-7.
Noguchi CT, et al. Alpha Thalassemia Changes Erythrocyte Heterogeneity in Sickle Cell Disease. J Clin Invest. 1985;75(5):1632-7. PubMed PMID: 2581999.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Alpha thalassemia changes erythrocyte heterogeneity in sickle cell disease. AU - Noguchi,C T, AU - Dover,G J, AU - Rodgers,G P, AU - Serjeant,G R, AU - Antonarakis,S E, AU - Anagnou,N P, AU - Higgs,D R, AU - Weatherall,D J, AU - Schechter,A N, PY - 1985/5/1/pubmed PY - 1985/5/1/medline PY - 1985/5/1/entrez SP - 1632 EP - 7 JF - The Journal of clinical investigation JO - J. Clin. Invest. VL - 75 IS - 5 N2 - Homozygous alpha-thalassemia has the beneficial effect in sickle cell anemia of reducing the hemolytic severity while changing several other hematological parameters. We examined in detail the cellular basis of some of these hematologic alterations. We find that the broad distribution in erythrocyte density and the large proportion of dense cells associated with sickle cell anemia are both reduced with coexisting alpha-thalassemia. Measurements of glycosylated hemoglobin levels as a function of cell density indicate that the accelerated increase in cell density, beyond normal cell aging, in sickle cell anemia is also reduced with alpha-thalassemia. The patients with homozygous alpha-thalassemia and sickle cell disease have slightly lower levels of hemoglobin F than the nonthalassemic sickle cell patients. Examination of hemoglobin F production revealed that the proportion of hemoglobin F containing reticulocytes remained unchanged, as did the proportion of hemoglobin F in cells containing hemoglobin F (F cells). Preferential survival of F cells occurs in sickle cell anemia, with or without alpha-thalassemia, and the slight difference in hemoglobin F levels appear to reflect differences in numbers of circulating F cells. Thus, in sickle cell disease with coexisting alpha-thalassemia, the change in the erythrocyte density profile, possibly due to inhibition of polymerization-related increases in cell density, explains the hematological improvement. SN - 0021-9738 UR - https://www.unboundmedicine.com/medline/citation/2581999/Alpha_thalassemia_changes_erythrocyte_heterogeneity_in_sickle_cell_disease_ L2 - https://doi.org/10.1172/JCI111870 DB - PRIME DP - Unbound Medicine ER -