Hairy cell leukemia (HCL) is a clonal expansion of abnormal memory-type B cells with specific features of activation, including the distinctive "hairy" morphology of the malignant cells that give the disease its name. The malignant hairy cells (HCs) display specific patterns of activated signaling components, express a spectrum of activation antigens and activated adhesion receptors, and spontaneously secrete a number of autocrine cytokines. Many recent studies clearly indicate that the pathology of HCL is, to a large extent, a direct reflection of this activated phenotype of the malignant cells.

Although HCL is a largely homogenous disease with respect to its cell-biological and clinicopathological features, no consistent abnormal cytogenetic profile has yet been demonstrated, and the nature of the onco-genic transformation of HCs remains unclear.

Here we first describe the specific cell-biological and molecular characteristics of HCs and discuss their importance for the distinctive clinicopathological features and therapy of HCL. We also present the currently known cytogenetics of this disease.

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