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Comparisons of oxygen gradient ektacytometry parameters between sickle cell patients with or without α-thalassaemia.
Br J Haematol. 2021 11; 195(4):629-633.BJ

Abstract

The present study tested the impact of α-thalassaemia on oxygen gradient ektacytometry in sickle cell anaemia (SCA). Three SCA groups were compared: (i) no α-thalassaemia (four α-genes, n = 62), (ii) silent α-thalassaemia (three α-genes, n = 35) and (iii) homozygous α-thalassaemia (two α-genes, n = 12). Red blood cell (RBC) deformability measured in normoxia was not different between the three groups. The lowest RBC deformability reached at low oxygen partial pressure (pO2) was greater and the pO2 at which RBC started to sickle was lower in the two α-genes group compared to the other groups. Our present study showed an effect of α-thalassaemia on oxygen gradient ektacytometry in SCA.

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Authors+Show Affiliations

Boisson C
Team 'Vascular Biology and Red Blood Cell', Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Université Claude Bernard Lyon 1, Université de Lyon, Villeurbanne, France. Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, Paris, France.
Renoux C
Team 'Vascular Biology and Red Blood Cell', Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Université Claude Bernard Lyon 1, Université de Lyon, Villeurbanne, France. Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, Paris, France. Laboratoire de Biochimie et de Biologie Moléculaire, UF de Biochimie des Pathologies Érythrocytaires, Centre de Biologie et de Pathologie Est, Hospices Civils de Lyon, Lyon, France.
Nader E
Team 'Vascular Biology and Red Blood Cell', Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Université Claude Bernard Lyon 1, Université de Lyon, Villeurbanne, France. Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, Paris, France.
Gauthier A
Team 'Vascular Biology and Red Blood Cell', Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Université Claude Bernard Lyon 1, Université de Lyon, Villeurbanne, France. Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, Paris, France. Institut d'Hématologie et d'Oncologie Pédiatrique, Hospices Civils de Lyon, Lyon, France.
Poutrel S
Team 'Vascular Biology and Red Blood Cell', Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Université Claude Bernard Lyon 1, Université de Lyon, Villeurbanne, France. Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, Paris, France. Département de Médecine Interne, Hôpital Edouard Herriot, Hospices Civils de Lyon, Lyon, France.
Rab M
Central Diagnostic Laboratory - Research, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands.
Fort R
Team 'Vascular Biology and Red Blood Cell', Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Université Claude Bernard Lyon 1, Université de Lyon, Villeurbanne, France. Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, Paris, France. Département de Médecine Interne, Hôpital Edouard Herriot, Hospices Civils de Lyon, Lyon, France.
Bertrand Y
Institut d'Hématologie et d'Oncologie Pédiatrique, Hospices Civils de Lyon, Lyon, France.
Stauffer E
Team 'Vascular Biology and Red Blood Cell', Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Université Claude Bernard Lyon 1, Université de Lyon, Villeurbanne, France. Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, Paris, France. Service d'Exploration Fonctionnelle Respiratoire et de Médecine du Sport, Hôpital Croix Rousse, Hospices Civils de Lyon, Lyon, France.
Cannas G
Département de Médecine Interne, Hôpital Edouard Herriot, Hospices Civils de Lyon, Lyon, France.
Kebaili K
Team 'Vascular Biology and Red Blood Cell', Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Université Claude Bernard Lyon 1, Université de Lyon, Villeurbanne, France. Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, Paris, France. Institut d'Hématologie et d'Oncologie Pédiatrique, Hospices Civils de Lyon, Lyon, France.
Virot E
Team 'Vascular Biology and Red Blood Cell', Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Université Claude Bernard Lyon 1, Université de Lyon, Villeurbanne, France. Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, Paris, France. Département de Médecine Interne, Hôpital Edouard Herriot, Hospices Civils de Lyon, Lyon, France.
Hot A
Département de Médecine Interne, Hôpital Edouard Herriot, Hospices Civils de Lyon, Lyon, France.
Sheehan V
Division of Hematology/Oncology, Department of Pediatrics, Baylor College of Medicine, Houston, TX, USA.
van Beers E
Van Creveldkliniek, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands.
van Wijk R
Central Diagnostic Laboratory - Research, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands.
Joly P
Team 'Vascular Biology and Red Blood Cell', Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Université Claude Bernard Lyon 1, Université de Lyon, Villeurbanne, France. Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, Paris, France. Laboratoire de Biochimie et de Biologie Moléculaire, UF de Biochimie des Pathologies Érythrocytaires, Centre de Biologie et de Pathologie Est, Hospices Civils de Lyon, Lyon, France.
Connes P
Team 'Vascular Biology and Red Blood Cell', Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Université Claude Bernard Lyon 1, Université de Lyon, Villeurbanne, France. Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, Paris, France.

MeSH

AdolescentAdultAnemia, Sickle CellChildChild, PreschoolErythrocyte DeformabilityErythrocyte IndicesGenotypeHumansOsmotic PressureOxygenShear StrengthYoung Adultalpha-Globinsalpha-Thalassemiabeta-Globins

Pub Type(s)

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

Language

eng

PubMed ID

34396507

Citation

Boisson, Camille, et al. "Comparisons of Oxygen Gradient Ektacytometry Parameters Between Sickle Cell Patients With or Without Α-thalassaemia." British Journal of Haematology, vol. 195, no. 4, 2021, pp. 629-633.
Boisson C, Renoux C, Nader E, et al. Comparisons of oxygen gradient ektacytometry parameters between sickle cell patients with or without α-thalassaemia. Br J Haematol. 2021;195(4):629-633.
Boisson, C., Renoux, C., Nader, E., Gauthier, A., Poutrel, S., Rab, M., Fort, R., Bertrand, Y., Stauffer, E., Cannas, G., Kebaili, K., Virot, E., Hot, A., Sheehan, V., van Beers, E., van Wijk, R., Joly, P., & Connes, P. (2021). Comparisons of oxygen gradient ektacytometry parameters between sickle cell patients with or without α-thalassaemia. British Journal of Haematology, 195(4), 629-633. https://doi.org/10.1111/bjh.17777
Boisson C, et al. Comparisons of Oxygen Gradient Ektacytometry Parameters Between Sickle Cell Patients With or Without Α-thalassaemia. Br J Haematol. 2021;195(4):629-633. PubMed PMID: 34396507.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Comparisons of oxygen gradient ektacytometry parameters between sickle cell patients with or without α-thalassaemia. AU - Boisson,Camille, AU - Renoux,Céline, AU - Nader,Elie, AU - Gauthier,Alexandra, AU - Poutrel,Solène, AU - Rab,Minke, AU - Fort,Romain, AU - Bertrand,Yves, AU - Stauffer,Emeric, AU - Cannas,Giovanna, AU - Kebaili,Kamila, AU - Virot,Emilie, AU - Hot,Arnaud, AU - Sheehan,Vivien, AU - van Beers,Eduard, AU - van Wijk,Richard, AU - Joly,Philippe, AU - Connes,Philippe, Y1 - 2021/08/15/ PY - 2021/07/27/revised PY - 2021/05/19/received PY - 2021/08/02/accepted PY - 2021/8/17/pubmed PY - 2021/12/24/medline PY - 2021/8/16/entrez KW - red blood cell deformability KW - sickle cell disease KW - sickling KW - α-thalassaemia SP - 629 EP - 633 JF - British journal of haematology JO - Br J Haematol VL - 195 IS - 4 N2 - The present study tested the impact of α-thalassaemia on oxygen gradient ektacytometry in sickle cell anaemia (SCA). Three SCA groups were compared: (i) no α-thalassaemia (four α-genes, n = 62), (ii) silent α-thalassaemia (three α-genes, n = 35) and (iii) homozygous α-thalassaemia (two α-genes, n = 12). Red blood cell (RBC) deformability measured in normoxia was not different between the three groups. The lowest RBC deformability reached at low oxygen partial pressure (pO2) was greater and the pO2 at which RBC started to sickle was lower in the two α-genes group compared to the other groups. Our present study showed an effect of α-thalassaemia on oxygen gradient ektacytometry in SCA. SN - 1365-2141 UR - https://www.unboundmedicine.com/medline/citation/34396507/Comparisons_of_oxygen_gradient_ektacytometry_parameters_between_sickle_cell_patients_with_or_without_α_thalassaemia_ DB - PRIME DP - Unbound Medicine ER -