• Haematologica · Nov 2012

    Clinical Trial

    Hemorheological risk factors of acute chest syndrome and painful vaso-occlusive crisis in children with sickle cell disease.

    • Yann Lamarre, Marc Romana, Xavier Waltz, Marie-Laure Lalanne-Mistrih, Benoît Tressières, Lydia Divialle-Doumdo, Marie-Dominique Hardy-Dessources, Jens Vent-Schmidt, Marie Petras, Cedric Broquere, Frederic Maillard, Vanessa Tarer, Maryse Etienne-Julan, and Philippe Connes.
    • Inserm U665, CHU de Pointe à Pitre, Hôpital Ricou, 97159 Pointe-à-Pitre, Guadeloupe. pconnes@yahoo.fr.
    • Haematologica. 2012 Nov 1;97(11):1641-7.

    BackgroundLittle 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 MethodsTo 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.ResultsMultivariate 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.ConclusionsOur 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.

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