The biology of hairy cells

The evidence that hairy cells are activated clonal late B cells is presented. The largely non-specific (i.e. not confined to hairy-cell leukaemia) chromosome and genetic abnormalities are then described. Next, the features of malignant-cell activation are considered, including the distinctive morphology of hairy cells and their expression of activation-related antigens and activated adhesion receptors. Also, signalling and cytokine production are discussed in the context of malignant-cell activation. It is then demonstrated that many of the distinctive clinicopathological features of hairy-cell leukaemia can be explained in terms of the interaction of the activated malignant cells with other types of cell and tissue matrix. Finally, the biological basis of the hairy cell's unusually high sensitivity to IFN-alpha and nucleoside analogues is discussed.