Pathogenetic determinants in Kawasaki disease: the haematological point of view.

Kawasaki disease is a multisystemic vasculitis that can result in coronary artery
lesions. It predominantly affects young children and is characterized by
prolonged fever, diffuse mucosal inflammation, indurative oedema of the hands and
feet, a polymorphous skin rash and non-suppurative lymphadenopathy. Coronary
artery involvement is the most important complication of Kawasaki disease and may
cause significant coronary stenosis resulting in ischemic heart disease. The
introduction of intravenous immunoglobulin decreases the incidence of coronary
artery lesions to less than 5%. The etiopathogenesis of this disease remains
unclear. Several lines of evidence suggest that an interplay between a microbial
infection and a genetic predisposition could take place in the development of the
disease. In this review, we summarize the state of the art of pathogenetic
mechanisms of Kawasaki disease underscoring the relevance of haematological
features as a novel field of investigation.