The endothelial biology of sickle cell disease: Inflammation and a chronic vasculopathy

被引:330
作者
Hebbel, RP
Osarogiagbon, R
Kaul, D
机构
[1] Univ Minnesota, Sch Med, Dept Med, Div Hematol Oncol Transplantat, Minneapolis, MN 55455 USA
[2] Univ Minnesota, Sch Med, VAsc Biol Ctr, Minneapolis, MN 55455 USA
[3] Vet Adm Ctr, Dept Med, Amarillo, TX USA
[4] Albert Einstein Coll Med, Dept Med, Div Hematol, Bronx, NY 10467 USA
关键词
endothelium; inflammation; sickle; vascular;
D O I
10.1080/10739680490278402
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
A single amino acid substitution in hemoglobin comprises the molecular basis for sickle cell anemia, but evolution of the corresponding clinical disease is extraordinarily complicated and likely involves multiple pathogenic factors. Sickle disease is fundamentally an inflammatory state, with activation of the endothelium, probably through proximate effects of reperfusion injury physiology and chronic molestation by adherent red cells and white cells. The disease also involves enhanced angiogenic propensity, activation of coagulation, disordered vasoregulation, and a component of chronic vasculopathy. Sickle cell anemia is truly an endothelial disease, and it is likely that genetic differences in endothelial function help govern its astonishing phenotypic diversity.
引用
收藏
页码:129 / 151
页数:23
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