The pathophysiology of the hairy cell

HCs are clonal late B cells that are related to memory cells and display specific features of activation. Many of the distinctive features of HCs (eg, morphology, TRAP) are related to this specific activation. Many of the distinctive histologic features of HCL can be related to constitutive production of cytokines (eg, FGF, fibrosis) and to the expression/activation of adhesion receptors (eg, alpha(4)beta(1), alpha(5)beta(1) and alpha(v)beta(3) integrins, CD44v3). HCs usually have mutated IGVH genes and have no consistent or specific chromosome abnormalities (5q additions and 7q deletions in a minority). The signals that are responsible for several of the phenotypic features of HCs have been identified, but the nature of the underlying oncogenic events remains unknown.