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

被引:89
作者
Lamarre, Yann [1 ]
Romana, Marc [1 ]
Waltz, Xavier [1 ]
Lalanne-Mistrih, Marie-Laure [1 ,2 ]
Tressleres, Benoit [2 ]
Divialle-Doumdo, Lydia [3 ]
Hardy-Dessources, Marie-Dominique [1 ]
Vent-Schmidt, Jens [1 ]
Petras, Marie [3 ]
Broquere, Cedric [1 ]
Maillard, Frederic [4 ]
Tarer, Vanessa [5 ]
Etienne-Julan, Maryse [3 ,5 ]
Connes, Philippe [1 ]
机构
[1] Univ Antilles Guyane, INSERM, U665, Pointe A Pitre, Guadeloupe, France
[2] Ctr Hosp Univ Pointe A Pitre, INSERM, CIC EC 802, Poine A Pitre, Guadeloupe, France
[3] Ctr Hosp Univ Pointe A Pitre, Unite Transversale Drepanocytose, Pointe A Pitre, Guadeloupe, France
[4] Ctr Hosp Univ Pointe A Pitre, Serv Pediat, Pointe a Pitre, Guadeloupe, France
[5] Ctr Hosp Univ Pointe A Pitre, Ctr Reference Malad Rare Drepanocytose Antilles G, Pointe a Pitre, Guadeloupe, France
来源
HAEMATOLOGICA-THE HEMATOLOGY JOURNAL | 2012年 / 97卷 / 11期
关键词
sickle cell anemia; hemoglobin SC disease; red blood aggregation; blood viscosity; red blood cell deformability; ENDOTHELIAL-CELLS; BLOOD; ANEMIA; DEFORMABILITY; AGGREGATION; SEVERITY; FLOW;
D O I
10.3324/haematol.2012.066670
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:1641 / 1647
页数:7
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