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 Mistrih Lalanne, Benoît Tressières, Lydia Divialle-Doumdo, Marie Dominique Dessources Hardy, Jens Vent-Schmidt, Marie Petras, Cedric Broquere, Frederic Maillard, Vanessa Tarer, Maryse Etienne-Julan, Philippe Connes

Research output: Contribution to journalReview articlepeer-review

71 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1641-1647
Number of pages7
JournalHaematologica
Volume97
Issue number11
DOIs
StatePublished - Nov 1 2012

Keywords

  • Blood viscosity
  • Hemoglobin SC disease
  • Red blood aggregation
  • Red blood cell deformability
  • Sickle cell anemia

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