NEW CONCEPTS IN ASSESSING SICKLE-CELL DISEASE SEVERITY

Vasoocclusion leads to pain, chronic organ damage, and a decreased lif e expectancy in patients with sickle cell disease. Therapeutic options for sickle cell disease have usually been evaluated according to thei r capacity for reducing the frequency of vasoocclusive crises requirin g clinical attention. However, the frequency of vasoocclusive crises i s not representative for the rate of accumulating organ damage in most sickle cell patients. This implies that the frequency of vasoocclusiv e crises needn't correlate with disease severity and, although being o f importance, cannot solely serve as a parameter of treatment efficacy . Therefore, additional new objective parameters are needed to effecti vely study the vasoocclusive process in sickle cell disease. Several s tudies show that intricate adhesive interactions between (red) blood c ells, plasma components, and endothelium play a crucial role in the pa thophysiology of sickle cell vasoocclusion, offering new potential par ameters to effectively assess disease severity as well as new therapeu tical targets in the near future, Whether these adhesive mechanisms in volve the causes or the effects of vasoocclusion will be determined it their inhibition, by interventive measures, results in therapeutic be nefits, (C) 1998 Wiley-Liss, Inc.