Tags

Type your tag names separated by a space and hit enter

Heterogeneity of sickle cell disease as shown by density profiles: effects of fetal hemoglobin and alpha thalassemia.
Haematologia (Budap). 1986; 19(3):177-84.H

Abstract

Factors that modify the intraerythrocytic concentration of hemoglobin S may influence the clinical expression of the disease. Using the phthalate ester method, the red blood cell density has been studied as a function of the mean corpuscular hemoglobin concentration. Four parameters have been used to compare the density distribution of the erythrocytes: D50 (median cell density), R60 (density range in which the middle 60% of the cells are found), F4 and F5 (proportion of cells with density greater than 1.110 and 1.120 g/ml). Compared to normal controls the density distribution of sickle red cells is heterogeneous, reproducible for the same patient (except in case of crisis), while different from one to another. The R60 is correlated with the percentage of dense cells, and the highest values for both R60 and dense cells are found when hemoglobin F is less than 10%. The highest values for the median cell density and dense red cells, but not for R60 which is normal, are observed in S/C patients. In sickle cell anemia patients, the median cell density values are not very different from the normal ones. The highest levels of hemoglobin F are found in this median subpopulation of red cells, while they are very low in the densest cells. R60 and the percentage of dense cells are not affected by the association of sickle cell disease with the deletion of one alpha gene. Their values are very near the normal ones in the case of an association with beta thalassemia or homozygous alpha thalassemia.(

ABSTRACT

TRUNCATED AT 250 WORDS)

Authors

No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

2430866

Citation

Baudin, V, et al. "Heterogeneity of Sickle Cell Disease as Shown By Density Profiles: Effects of Fetal Hemoglobin and Alpha Thalassemia." Haematologia, vol. 19, no. 3, 1986, pp. 177-84.
Baudin V, Pagnier J, Labie D, et al. Heterogeneity of sickle cell disease as shown by density profiles: effects of fetal hemoglobin and alpha thalassemia. Haematologia (Budap). 1986;19(3):177-84.
Baudin, V., Pagnier, J., Labie, D., Girot, R., & Wajcman, H. (1986). Heterogeneity of sickle cell disease as shown by density profiles: effects of fetal hemoglobin and alpha thalassemia. Haematologia, 19(3), 177-84.
Baudin V, et al. Heterogeneity of Sickle Cell Disease as Shown By Density Profiles: Effects of Fetal Hemoglobin and Alpha Thalassemia. Haematologia (Budap). 1986;19(3):177-84. PubMed PMID: 2430866.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Heterogeneity of sickle cell disease as shown by density profiles: effects of fetal hemoglobin and alpha thalassemia. AU - Baudin,V, AU - Pagnier,J, AU - Labie,D, AU - Girot,R, AU - Wajcman,H, PY - 1986/1/1/pubmed PY - 1986/1/1/medline PY - 1986/1/1/entrez SP - 177 EP - 84 JF - Haematologia JO - Haematologia (Budap) VL - 19 IS - 3 N2 - Factors that modify the intraerythrocytic concentration of hemoglobin S may influence the clinical expression of the disease. Using the phthalate ester method, the red blood cell density has been studied as a function of the mean corpuscular hemoglobin concentration. Four parameters have been used to compare the density distribution of the erythrocytes: D50 (median cell density), R60 (density range in which the middle 60% of the cells are found), F4 and F5 (proportion of cells with density greater than 1.110 and 1.120 g/ml). Compared to normal controls the density distribution of sickle red cells is heterogeneous, reproducible for the same patient (except in case of crisis), while different from one to another. The R60 is correlated with the percentage of dense cells, and the highest values for both R60 and dense cells are found when hemoglobin F is less than 10%. The highest values for the median cell density and dense red cells, but not for R60 which is normal, are observed in S/C patients. In sickle cell anemia patients, the median cell density values are not very different from the normal ones. The highest levels of hemoglobin F are found in this median subpopulation of red cells, while they are very low in the densest cells. R60 and the percentage of dense cells are not affected by the association of sickle cell disease with the deletion of one alpha gene. Their values are very near the normal ones in the case of an association with beta thalassemia or homozygous alpha thalassemia.(ABSTRACT TRUNCATED AT 250 WORDS) SN - 0017-6559 UR - https://www.unboundmedicine.com/medline/citation/2430866/Heterogeneity_of_sickle_cell_disease_as_shown_by_density_profiles:_effects_of_fetal_hemoglobin_and_alpha_thalassemia_ L2 - http://www.diseaseinfosearch.org/result/333 DB - PRIME DP - Unbound Medicine ER -