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Publications

Journal Article Epublish 12 citations

PubMed: 33916502 DOI: 10.3390/cells10040811

Effects of Genotypes and Treatment on Oxygenscan Parameters in Sickle Cell Disease.

Cells 10(4)

Authors: Camille Boisson, Minke A E Rab, Elie Nader, Céline Renoux, Celeste Kanne, Jennifer Bos, Brigitte A van Oirschot, Philippe Joly, Romain Fort, Alexandra Gauthier, Emeric Stauffer, Solene Poutrel, Kamila Kebaili, Giovanna Cannas, Nathalie Garnier, Cécile Renard, Olivier Hequet, Arnaud Hot, Yves Bertrand, Richard van Wijk, Vivien A Sheehan, Eduard J van Beers, Philippe Connes

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.
  • Department of Pediatrics, Division of Hematology/Oncology, Baylor College of Medicine, Houston, TX 77030, USA.
  • 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.
  • Centre de Médecine du Sommeil et des Maladies Respiratoires, Hôpital Croix Rousse, Hospices Civils de Lyon, 69004 Lyon, France.
  • Apheresis Unit, Etablissement Français du Sang Rhône Alpes, Centre Hospitalier Lyon Sud Pierre Bénite, 69310 Pierre Bénite, France.

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.

MeSH

Erythrocytes, Anemia, Sickle Cell, Genotype, Hydroxyurea, Blood Cells, Therapeutics

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