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Hemorheological risk factors of acute chest syndrome and painful vaso-occlusive crisis in children with sickle cell disease. ; Hemorheological risk factors of acute chest syndrome and painful vaso-occlusive crisis in children with sickle cell disease. : Blood rheology in sickle cell disease
Auteur(s) : Lamarre, Yann Romana, Marc Waltz, Xavier Lalanne-Mistrih, Marie-Laure Tressières, Benoît Divialle-Doumdo, Lydia Hardy-Dessources, Marie-Dominique Vent-Schmidt, Jens
Auteurs secondaires : Protéines de la membrane érythrocytaire et homologues non-érythroides ; Université des Antilles et de la Guyane (UAG) - Institut National de la Transfusion Sanguine [Paris] (INTS) - Université Paris Diderot - Paris 7 (UPD7) - Université de la Réunion (UR) - Institut National de la Santé et de la Recherche Médicale (INSERM) Centre d'investigation clinique Antilles-Guyane ; Institut National de la Santé et de la Recherche Médicale (INSERM) - CH Cayenne Unité Transversale de la Drépanocytose ; CHU de Pointe-à-Pitre/Abymes Service de Pédiatrie ; CHU de Pointe-à-Pitre/Abymes Service d'Orthopédie et de Traumatologie ; Université des Antilles et de la Guyane (UAG) - CHU Pointe-à-Pitre/Abymes

Éditeur(s) : HAL CCSD Ferrata Storti Foundation
Résumé : International audience
BACKGROUND: Little is known about the effects of blood rheology on the occurrence of acute chest syndrome and painful vaso-occlusive crises in children with sickle cell anemia and hemoglobin SC disease. DESIGN AND METHODS: To address this issue, steady-state hemorheological profiles (blood viscosity, red blood cell deformability, aggregation properties) and hematologic parameters were assessed in 44 children with sickle cell anemia and 49 children with hemoglobin SC disease (8-16 years old) followed since birth. Clinical charts were retrospectively reviewed to determine prior acute chest syndrome or vaso-occlusive episodes, and rates of these complications were calculated. RESULTS: Multivariate analysis revealed that: 1) a higher steady-state blood viscosity was associated with a higher rate of vaso-occlusive crises in children with sickle cell anemia, but not in children with hemoglobin SC disease; 2) a higher steady-state red blood cell disaggregation threshold was associated with previous history of acute chest syndrome in children with hemoglobin SC disease and boys with sickle cell anemia. CONCLUSIONS: Our results indicate for the first time that the red blood cell aggregation properties may play a role in the pathophysiology of acute chest syndrome in children with hemoglobin SC disease and boys with sickle cell anemia. In addition, whereas greater blood viscosity is associated with a higher rate of vaso-occlusive crises in children with sickle cell anemia, no association was found in children with hemoglobin SC disease, underscoring differences in the etiology of vaso-occlusive crises between sickle cell anemia and hemoglobin SC disease.
ISSN: 0390-6078

inserm-00849038
http://www.hal.inserm.fr/inserm-00849038
http://www.hal.inserm.fr/inserm-00849038/document
http://www.hal.inserm.fr/inserm-00849038/file/inserm-00849038.pdf
DOI : 10.3324/haematol.2012.066670

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