B-cell receptor signalling in B-cell lymphoproliferative disorders

B- cell chronic lymphoproliferative disorders are a heterogeneous group of mature B neoplasms characterized by constitutive activation of the B cell receptor (BCR) signaling, that is essential in the pathophysiological process. The mechanisms of activation of the pathway are variable and often rely on a chronic antigenic stimulation or on mutations in pathway effectors responsible for the constitutive activation of signaling components and transcription factors including NF-.B. The BCR is at the crossroad with other signaling pathways essential for the survival and proliferation of the tumor clone. Understanding the role of the BCR pathway has enabled the development of therapeutic approaches based on the targeting of signaling intermediates.