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Measurements of red cell deformability and hydration reflect HbF and HbA2 in blood from patients with sickle cell anemia.
Blood Cells Mol Dis. 2017 06; 65:41-50.BC

Abstract

Decreased erythrocyte deformability, as measured by ektacytometry, may be associated with disease severity in sickle cell anemia (SCA). Heterogeneous populations of rigid and deformable cells in SCA blood result in distortions of diffraction pattern measurements that correlate with the concentration of hemoglobin S (HbS) and the percentage of irreversibly sickled cells. We hypothesize that red cell heterogeneity, as well as deformability, will also be influenced by the concentration of alternative hemoglobins such as fetal hemoglobin (HbF) and the adult variant, HbA2. To test this hypothesis, we investigate the relationship between diffraction pattern distortion, osmotic gradient ektacytometry parameters, and the hemoglobin composition of SCA blood. We observe a correlation between the extent of diffraction pattern distortions and percentage of HbF and HbA2. Osmotic gradient ektacytometry data indicate that minimum elongation in the hypotonic region is positively correlated with HbF, as is the osmolality at which it occurs. The osmolality at both minimum and maximum elongation is inversely correlated with HbS and HbA2. These data suggest that HbF may effectively improve surface-to-volume ratio and osmotic fragility in SCA erythrocytes. HbA2 may be relatively ineffective in improving these characteristics or cellular hydration at the levels found in this patient cohort.

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Authors+Show Affiliations

Parrow NL
Molecular and Clinical Nutrition Section, Digestive Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, MD, USA.
Tu H
Molecular and Clinical Nutrition Section, Digestive Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, MD, USA.
Nichols J
Molecular and Clinical Hematology Branch, National Heart, Lung and Blood Institute, Bethesda, MD, USA.
Violet PC
Molecular and Clinical Nutrition Section, Digestive Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, MD, USA.
Pittman CA
Sickle Cell Branch, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD, USA.
Fitzhugh C
Sickle Cell Branch, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD, USA.
Fleming RE
Department of Pediatrics, Saint Louis University School of Medicine, St Louis, MO, USA; Edward A. Doisy Department of Biochemistry and Molecular Biology, Saint Louis University School of Medicine, St Louis, MO, USA.
Mohandas N
Red Cell Physiology Laboratory, New York Blood Center, New York, NY, USA.
Tisdale JF
Molecular and Clinical Hematology Branch, National Heart, Lung and Blood Institute, Bethesda, MD, USA.
Levine M
Molecular and Clinical Nutrition Section, Digestive Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, MD, USA. Electronic address: markl@intra.niddk.nih.gov.

MeSH

AdultAnemia, Sickle CellAntisickling AgentsBlood Cell CountBlood TransfusionErythrocyte DeformabilityErythrocyte IndicesFemaleFetal HemoglobinHemoglobin, SickleHumansHydroxyureaMaleMiddle AgedOsmotic FragilityYoung Adult

Pub Type(s)

Journal Article
Research Support, N.I.H., Intramural

Language

eng

PubMed ID

28472705

Citation

Parrow, Nermi L., et al. "Measurements of Red Cell Deformability and Hydration Reflect HbF and HbA2 in Blood From Patients With Sickle Cell Anemia." Blood Cells, Molecules & Diseases, vol. 65, 2017, pp. 41-50.
Parrow NL, Tu H, Nichols J, et al. Measurements of red cell deformability and hydration reflect HbF and HbA2 in blood from patients with sickle cell anemia. Blood Cells Mol Dis. 2017;65:41-50.
Parrow, N. L., Tu, H., Nichols, J., Violet, P. C., Pittman, C. A., Fitzhugh, C., Fleming, R. E., Mohandas, N., Tisdale, J. F., & Levine, M. (2017). Measurements of red cell deformability and hydration reflect HbF and HbA2 in blood from patients with sickle cell anemia. Blood Cells, Molecules & Diseases, 65, 41-50. https://doi.org/10.1016/j.bcmd.2017.04.005
Parrow NL, et al. Measurements of Red Cell Deformability and Hydration Reflect HbF and HbA2 in Blood From Patients With Sickle Cell Anemia. Blood Cells Mol Dis. 2017;65:41-50. PubMed PMID: 28472705.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Measurements of red cell deformability and hydration reflect HbF and HbA2 in blood from patients with sickle cell anemia. AU - Parrow,Nermi L, AU - Tu,Hongbin, AU - Nichols,James, AU - Violet,Pierre-Christian, AU - Pittman,Corinne A, AU - Fitzhugh,Courtney, AU - Fleming,Robert E, AU - Mohandas,Narla, AU - Tisdale,John F, AU - Levine,Mark, Y1 - 2017/04/18/ PY - 2017/04/04/received PY - 2017/04/14/accepted PY - 2017/5/5/pubmed PY - 2018/3/27/medline PY - 2017/5/5/entrez KW - Erythrocytes KW - Fetal hemoglobin KW - Sickle cell anemia KW - Sickle cell disease SP - 41 EP - 50 JF - Blood cells, molecules & diseases JO - Blood Cells Mol Dis VL - 65 N2 - Decreased erythrocyte deformability, as measured by ektacytometry, may be associated with disease severity in sickle cell anemia (SCA). Heterogeneous populations of rigid and deformable cells in SCA blood result in distortions of diffraction pattern measurements that correlate with the concentration of hemoglobin S (HbS) and the percentage of irreversibly sickled cells. We hypothesize that red cell heterogeneity, as well as deformability, will also be influenced by the concentration of alternative hemoglobins such as fetal hemoglobin (HbF) and the adult variant, HbA2. To test this hypothesis, we investigate the relationship between diffraction pattern distortion, osmotic gradient ektacytometry parameters, and the hemoglobin composition of SCA blood. We observe a correlation between the extent of diffraction pattern distortions and percentage of HbF and HbA2. Osmotic gradient ektacytometry data indicate that minimum elongation in the hypotonic region is positively correlated with HbF, as is the osmolality at which it occurs. The osmolality at both minimum and maximum elongation is inversely correlated with HbS and HbA2. These data suggest that HbF may effectively improve surface-to-volume ratio and osmotic fragility in SCA erythrocytes. HbA2 may be relatively ineffective in improving these characteristics or cellular hydration at the levels found in this patient cohort. SN - 1096-0961 UR - https://www.unboundmedicine.com/medline/citation/28472705/Measurements_of_red_cell_deformability_and_hydration_reflect_HbF_and_HbA2_in_blood_from_patients_with_sickle_cell_anemia_ DB - PRIME DP - Unbound Medicine ER -