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New concepts in assessing sickle cell disease severity

✍ Scribed by Schnog, John-John B.; Lard, Leroy R.; Rojer, Robert A.; Van der Dijs, Fey P. L.; Muskiet, Frits A. J.; Duits, Ashley J.

Publisher: John Wiley and Sons
Year: 1998
Tongue: English
Weight: 39 KB
Volume: 58
Category: Article
ISSN: 0361-8609
DOI: 10.1002/(sici)1096-8652(199805)58:1<61::aid-ajh11>3.0.co;2-8

No coin nor oath required. For personal study only.

✦ Synopsis

Vasoocclusion leads to pain, chronic organ damage, and a decreased life expectancy in patients with sickle cell disease. Therapeutic options for sickle cell disease have usually been evaluated according to their capacity for reducing the frequency of vasoocclusive crises requiring clinical attention. However, the frequency of vasoocclusive crises is not representative for the rate of accumulating organ damage in most sickle cell patients. This implies that the frequency of vasoocclusive crises needn't correlate with disease severity and, although being of importance, cannot solely serve as a parameter of treatment efficacy. Therefore, additional new objective parameters are needed to effectively study the vasoocclusive process in sickle cell disease. Several studies show that intricate adhesive interactions between (red) blood cells, plasma components, and endothelium play a crucial role in the pathophysiology of sickle cell vasoocclusion, offering new potential parameters to effectively assess disease severity as well as new therapeutical targets in the near future. Whether these adhesive mechanisms involve the causes or the effects of vasoocclusion will be determined if their inhibition, by interventive measures, results in therapeutic benefits. Am.

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