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Rheological properties of sickle erythrocytes in patients with sickle-cell anemia: The effect of hydroxyurea, fetal hemoglobin, and α-thalassemia.
Eur J Haematol. 2018 Dec; 101(6):798-803.EJ

Abstract

OBJECTIVE

Determine the effect of fetal hemoglobin (HbF) and α-thalassemia on red blood cell (RBC) deformability of patients with sickle-cell anemia (SCA) with and without hydroxyurea (HU).

METHODS

Adult patients were enrolled in the Sickle Cell Program of the Cardeza Foundation (Thomas Jefferson University) and were followed up prospectively during the period in which the Multicenter Study of Hydroxyurea (MSH) in patients with SCA was conducted. Ninety-one patients did not receive HU, 20 patients were enrolled in MSH, and 10 patients were enrolled in an open-label study of HU in SCA. Of the 20 patients enrolled in MSH, 11 took HU and nine took placebo. Control group included 113 normal individuals. Red blood cell deformability index (DI) was measured by ektacytometry.

RESULTS

Patients with SCA taking HU (n = 21) had higher DI than those taking placebo (n = 9) or who were not taking this therapy (n = 91). In patients without therapy, those with α-thalassemia (n = 31) had higher DI than those without. We showed a significant positive correlation between the level of HbF and DI. SCA patients without α-thalassemia and HbF <10% (n = 48) had lower DI than patients with α-thalassemia and HbF <10% (n = 23) and patients with (n = 8) or without α-thalassemia but with HbF >10% (n = 12). DI measured in patients without α-thalassemia and HbF >10% was higher than in the three other subgroups.

CONCLUSION

Elevated levels of HbF with or without HU and α-thalassemia improve sickle RBC rheology, which, in turn, improve the clinical picture of SCA.

Links

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Authors+Show Affiliations

Ballas SK
Department of Medicine, Cardeza Foundation for Hematologic Research, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania.
Connes P
Laboratoire LIBM EA7424, Equipe "Biologie Vasculaire et du Globule Rouge", Université Claude Bernard Lyon 1, Villeurbanne, France. Laboratoire d'Excellence du Globule Rouge (LABEX GR-Ex), PRES Sorbonne, Paris, France. Institut Universitaire de France (IUF), Paris, France.
Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia
No affiliation info available

MeSH

Anemia, Sickle CellBiomarkersErythrocyte IndicesErythrocytes, AbnormalFetal HemoglobinGenotypeHemorheologyHumansHydroxyureaTreatment Outcomealpha-Globinsalpha-Thalassemia

Pub Type(s)

Journal Article

Language

eng

PubMed ID

30204261

Citation

Ballas, Samir K., et al. "Rheological Properties of Sickle Erythrocytes in Patients With Sickle-cell Anemia: the Effect of Hydroxyurea, Fetal Hemoglobin, and Α-thalassemia." European Journal of Haematology, vol. 101, no. 6, 2018, pp. 798-803.
Ballas SK, Connes P, Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. Rheological properties of sickle erythrocytes in patients with sickle-cell anemia: The effect of hydroxyurea, fetal hemoglobin, and α-thalassemia. Eur J Haematol. 2018;101(6):798-803.
Ballas, S. K., & Connes, P. (2018). Rheological properties of sickle erythrocytes in patients with sickle-cell anemia: The effect of hydroxyurea, fetal hemoglobin, and α-thalassemia. European Journal of Haematology, 101(6), 798-803. https://doi.org/10.1111/ejh.13173
Ballas SK, Connes P, Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. Rheological Properties of Sickle Erythrocytes in Patients With Sickle-cell Anemia: the Effect of Hydroxyurea, Fetal Hemoglobin, and Α-thalassemia. Eur J Haematol. 2018;101(6):798-803. PubMed PMID: 30204261.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Rheological properties of sickle erythrocytes in patients with sickle-cell anemia: The effect of hydroxyurea, fetal hemoglobin, and α-thalassemia. AU - Ballas,Samir K, AU - Connes,Philippe, AU - ,, Y1 - 2018/10/09/ PY - 2018/05/31/received PY - 2018/08/24/revised PY - 2018/08/27/accepted PY - 2018/9/12/pubmed PY - 2019/2/12/medline PY - 2018/9/12/entrez KW - hydroxyurea KW - red blood cell deformability KW - sickle-cell anemia KW - α-thalassemia SP - 798 EP - 803 JF - European journal of haematology JO - Eur J Haematol VL - 101 IS - 6 N2 - OBJECTIVE: Determine the effect of fetal hemoglobin (HbF) and α-thalassemia on red blood cell (RBC) deformability of patients with sickle-cell anemia (SCA) with and without hydroxyurea (HU). METHODS: Adult patients were enrolled in the Sickle Cell Program of the Cardeza Foundation (Thomas Jefferson University) and were followed up prospectively during the period in which the Multicenter Study of Hydroxyurea (MSH) in patients with SCA was conducted. Ninety-one patients did not receive HU, 20 patients were enrolled in MSH, and 10 patients were enrolled in an open-label study of HU in SCA. Of the 20 patients enrolled in MSH, 11 took HU and nine took placebo. Control group included 113 normal individuals. Red blood cell deformability index (DI) was measured by ektacytometry. RESULTS: Patients with SCA taking HU (n = 21) had higher DI than those taking placebo (n = 9) or who were not taking this therapy (n = 91). In patients without therapy, those with α-thalassemia (n = 31) had higher DI than those without. We showed a significant positive correlation between the level of HbF and DI. SCA patients without α-thalassemia and HbF <10% (n = 48) had lower DI than patients with α-thalassemia and HbF <10% (n = 23) and patients with (n = 8) or without α-thalassemia but with HbF >10% (n = 12). DI measured in patients without α-thalassemia and HbF >10% was higher than in the three other subgroups. CONCLUSION: Elevated levels of HbF with or without HU and α-thalassemia improve sickle RBC rheology, which, in turn, improve the clinical picture of SCA. SN - 1600-0609 UR - https://www.unboundmedicine.com/medline/citation/30204261/Rheological_properties_of_sickle_erythrocytes_in_patients_with_sickle_cell_anemia:_The_effect_of_hydroxyurea_fetal_hemoglobin_and_��_thalassemia_ DB - PRIME DP - Unbound Medicine ER -