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


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.
Inserm U665, CHU de Pointe à Pitre, Hôpital Ricou, 97159 Pointe-à-Pitre, Guadeloupe.  pconnes@yahoo.fr.
Yann
Y
Lamarre
Marc
M
Romana
Xavier
X
Waltz
Marie-Laure
ML
Lalanne-Mistrih
Benoît
B
Tressières
Lydia
L
Divialle-Doumdo
Marie-Dominique
MD
Hardy-Dessources
Jens
J
Vent-Schmidt
Marie
M
Petras
Cedric
C
Broquere
Frederic
F
Maillard
Vanessa
V
Tarer
Maryse
M
Etienne-Julan
Philippe
P
Connes
D056586:Acute Chest Syndrome / Q000097:blood*; D000293:Adolescent; D001809:Blood Viscosity; D002648:Child; D003251:Constriction, Pathologic / Q000097:blood; D004903:Erythrocyte Aggregation; D004907:Erythrocyte Deformability; D005260:Female; D006801:Humans; D008297:Male; D010146:Pain / Q000097:blood*; D012189:Retrospective Studies; D012307:Risk Factors