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Effects of Genotypes and Treatment on Oxygenscan Parameters in Sickle Cell Disease.
Cells. 2021 04 05; 10(4)C

Abstract

(1) Background: The aim of the present study was to compare oxygen gradient ektacytometry parameters between sickle cell patients of different genotypes (SS, SC, and S/β+) or under different treatments (hydroxyurea or chronic red blood cell exchange). (2) Methods: Oxygen gradient ektacytometry was performed in 167 adults and children at steady state. In addition, five SS patients had oxygenscan measurements at steady state and during an acute complication requiring hospitalization. (3) Results: Red blood cell (RBC) deformability upon deoxygenation (EImin) and in normoxia (EImax) was increased, and the susceptibility of RBC to sickle upon deoxygenation was decreased in SC patients when compared to untreated SS patients older than 5 years old. SS patients under chronic red blood cell exchange had higher EImin and EImax and lower susceptibility of RBC to sickle upon deoxygenation compared to untreated SS patients, SS patients younger than 5 years old, and hydroxyurea-treated SS and SC patients. The susceptibility of RBC to sickle upon deoxygenation was increased in the five SS patients during acute complication compared to steady state, although the difference between steady state and acute complication was variable from one patient to another. (4) Conclusions: The present study demonstrates that oxygen gradient ektacytometry parameters are affected by sickle cell disease (SCD) genotype and treatment.

Authors+Show Affiliations

Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Team « Vascular Biology and Red Blood Cell », Université Claude Bernard Lyon 1, Université de Lyon, 69008 Lyon, France. Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, 75006 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, 69500 Bron, France.Central Diagnostic Laboratory-Research, University Medical Center Utrecht, Utrecht University, 85500, 3508 GA Utrecht, The Netherlands. Van Creveldkliniek, University Medical Center Utrecht, Utrecht University, 85500, 3508 GA Utrecht, The Netherlands.Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Team « Vascular Biology and Red Blood Cell », Université Claude Bernard Lyon 1, Université de Lyon, 69008 Lyon, France. Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, 75006 Paris, France.Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Team « Vascular Biology and Red Blood Cell », Université Claude Bernard Lyon 1, Université de Lyon, 69008 Lyon, France. Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, 75006 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, 69500 Bron, France.Department of Pediatrics, Division of Hematology/Oncology, Baylor College of Medicine, Houston, TX 77030, USA.Central Diagnostic Laboratory-Research, University Medical Center Utrecht, Utrecht University, 85500, 3508 GA Utrecht, The Netherlands.Central Diagnostic Laboratory-Research, University Medical Center Utrecht, Utrecht University, 85500, 3508 GA Utrecht, The Netherlands.Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Team « Vascular Biology and Red Blood Cell », Université Claude Bernard Lyon 1, Université de Lyon, 69008 Lyon, France. Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, 75006 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, 69500 Bron, France.Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Team « Vascular Biology and Red Blood Cell », Université Claude Bernard Lyon 1, Université de Lyon, 69008 Lyon, France. Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, 75006 Paris, France. Département de Médecine Interne, Hôpital Edouard Herriot, Hospices Civils de Lyon, 69008 Lyon, France.Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Team « Vascular Biology and Red Blood Cell », Université Claude Bernard Lyon 1, Université de Lyon, 69008 Lyon, France. Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, 75006 Paris, France. Institut d'Hématologie et d'Oncologie Pédiatrique, Hospices Civils de Lyon, 69008 Lyon, France.Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Team « Vascular Biology and Red Blood Cell », Université Claude Bernard Lyon 1, Université de Lyon, 69008 Lyon, France. Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, 75006 Paris, France. Centre de Médecine du Sommeil et des Maladies Respiratoires, Hôpital Croix Rousse, Hospices Civils de Lyon, 69004 Lyon, France.Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Team « Vascular Biology and Red Blood Cell », Université Claude Bernard Lyon 1, Université de Lyon, 69008 Lyon, France. Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, 75006 Paris, France. Département de Médecine Interne, Hôpital Edouard Herriot, Hospices Civils de Lyon, 69008 Lyon, France.Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Team « Vascular Biology and Red Blood Cell », Université Claude Bernard Lyon 1, Université de Lyon, 69008 Lyon, France. Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, 75006 Paris, France. Institut d'Hématologie et d'Oncologie Pédiatrique, Hospices Civils de Lyon, 69008 Lyon, France.Département de Médecine Interne, Hôpital Edouard Herriot, Hospices Civils de Lyon, 69008 Lyon, France.Institut d'Hématologie et d'Oncologie Pédiatrique, Hospices Civils de Lyon, 69008 Lyon, France.Institut d'Hématologie et d'Oncologie Pédiatrique, Hospices Civils de Lyon, 69008 Lyon, France.Apheresis Unit, Etablissement Français du Sang Rhône Alpes, Centre Hospitalier Lyon Sud Pierre Bénite, 69310 Pierre Bénite, France.Département de Médecine Interne, Hôpital Edouard Herriot, Hospices Civils de Lyon, 69008 Lyon, France.Institut d'Hématologie et d'Oncologie Pédiatrique, Hospices Civils de Lyon, 69008 Lyon, France.Central Diagnostic Laboratory-Research, University Medical Center Utrecht, Utrecht University, 85500, 3508 GA Utrecht, The Netherlands.Department of Pediatrics, Division of Hematology/Oncology, Baylor College of Medicine, Houston, TX 77030, USA.Van Creveldkliniek, University Medical Center Utrecht, Utrecht University, 85500, 3508 GA Utrecht, The Netherlands.Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Team « Vascular Biology and Red Blood Cell », Université Claude Bernard Lyon 1, Université de Lyon, 69008 Lyon, France. Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, 75006 Paris, France.

Pub Type(s)

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

Language

eng

PubMed ID

33916502

Citation

Boisson, Camille, et al. "Effects of Genotypes and Treatment On Oxygenscan Parameters in Sickle Cell Disease." Cells, vol. 10, no. 4, 2021.
Boisson C, Rab MAE, Nader E, et al. Effects of Genotypes and Treatment on Oxygenscan Parameters in Sickle Cell Disease. Cells. 2021;10(4).
Boisson, C., Rab, M. A. E., Nader, E., Renoux, C., Kanne, C., Bos, J., van Oirschot, B. A., Joly, P., Fort, R., Gauthier, A., Stauffer, E., Poutrel, S., Kebaili, K., Cannas, G., Garnier, N., Renard, C., Hequet, O., Hot, A., Bertrand, Y., ... Connes, P. (2021). Effects of Genotypes and Treatment on Oxygenscan Parameters in Sickle Cell Disease. Cells, 10(4). https://doi.org/10.3390/cells10040811
Boisson C, et al. Effects of Genotypes and Treatment On Oxygenscan Parameters in Sickle Cell Disease. Cells. 2021 04 5;10(4) PubMed PMID: 33916502.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Effects of Genotypes and Treatment on Oxygenscan Parameters in Sickle Cell Disease. AU - Boisson,Camille, AU - Rab,Minke A E, AU - Nader,Elie, AU - Renoux,Céline, AU - Kanne,Celeste, AU - Bos,Jennifer, AU - van Oirschot,Brigitte A, AU - Joly,Philippe, AU - Fort,Romain, AU - Gauthier,Alexandra, AU - Stauffer,Emeric, AU - Poutrel,Solene, AU - Kebaili,Kamila, AU - Cannas,Giovanna, AU - Garnier,Nathalie, AU - Renard,Cécile, AU - Hequet,Olivier, AU - Hot,Arnaud, AU - Bertrand,Yves, AU - van Wijk,Richard, AU - Sheehan,Vivien A, AU - van Beers,Eduard J, AU - Connes,Philippe, Y1 - 2021/04/05/ PY - 2021/03/08/received PY - 2021/03/25/revised PY - 2021/03/30/accepted PY - 2021/4/30/entrez PY - 2021/5/1/pubmed PY - 2021/10/21/medline KW - acute complication KW - clinical severity KW - oxygenscan KW - red blood cell deformability KW - sickle cell disease JF - Cells JO - Cells VL - 10 IS - 4 N2 - (1) Background: The aim of the present study was to compare oxygen gradient ektacytometry parameters between sickle cell patients of different genotypes (SS, SC, and S/β+) or under different treatments (hydroxyurea or chronic red blood cell exchange). (2) Methods: Oxygen gradient ektacytometry was performed in 167 adults and children at steady state. In addition, five SS patients had oxygenscan measurements at steady state and during an acute complication requiring hospitalization. (3) Results: Red blood cell (RBC) deformability upon deoxygenation (EImin) and in normoxia (EImax) was increased, and the susceptibility of RBC to sickle upon deoxygenation was decreased in SC patients when compared to untreated SS patients older than 5 years old. SS patients under chronic red blood cell exchange had higher EImin and EImax and lower susceptibility of RBC to sickle upon deoxygenation compared to untreated SS patients, SS patients younger than 5 years old, and hydroxyurea-treated SS and SC patients. The susceptibility of RBC to sickle upon deoxygenation was increased in the five SS patients during acute complication compared to steady state, although the difference between steady state and acute complication was variable from one patient to another. (4) Conclusions: The present study demonstrates that oxygen gradient ektacytometry parameters are affected by sickle cell disease (SCD) genotype and treatment. SN - 2073-4409 UR - https://www.unboundmedicine.com/medline/citation/33916502/Effects_of_Genotypes_and_Treatment_on_Oxygenscan_Parameters_in_Sickle_Cell_Disease_ DB - PRIME DP - Unbound Medicine ER -