Cytokines and plasma factors in sickle cell disease

Plasma cytokines and related factors represent a burgeoning area of inquiry related to the pathogenesis in sickle cell disease. Cytokines derived from platelets, white blood cells and endothelial cells have all been implicated in the development of several sequelae of this disease. In this review, we seek to provide an overview of the noted and potentially novel roles for several key plasma factors in sickle cell disease. We also consider the putative role for those cytokines implicated by genetic analysis in sickle cell disease, but where the pathogenic, or ameliorative, role has yet to be determined.

New roles for the platelet as a key mediator in the release of cytokines in sickle cell disease have recently been demonstrated. Angiogenic and inflammatory factors are also being explored in this illness. Members of the vascular endothelial growth factor and transforming growth factor-beta superfamilies have been suggested to contribute to several key events in pathogenesis of sickle cell disease, but with the promise of nitrous oxide therapy in this disorder, these cytokines merit a fresh perspective in the context of sickle cell disease.

Increased understanding of the origin and pathology of cytokine levels in sickle cell disease may provide novel therapeutic approaches in the management of the disease.