title: Intrinsic and extrinsic factors influencing the clinical course of B-cell chronic lymphocytic leukemia: prognostic markers with pathogenetic relevance.
abstract: B-cell chronic lymphocytic leukemia (CLL), the most frequent leukemia in the Western world, is characterized by extremely variable clinical courses with survivals ranging from 1 to more than 15 years. The pathogenetic factors playing a key role in defining the biological features of CLL cells, hence eventually influencing the clinical aggressiveness of the disease, are here divided into "intrinsic factors", mainly genomic alterations of CLL cells, and "extrinsic factors", responsible for direct microenvironmental interactions of CLL cells; the latter group includes interactions of CLL cells occurring via the surface B cell receptor (BCR) and dependent to specific molecular features of the BCR itself and/or to the presence of the BCR-associated molecule ZAP-70, or via other non-BCR-dependent interactions, e.g. specific receptor/ligand interactions, such as CD38/CD31 or CD49d/VCAM-1. A putative final model, discussing the pathogenesis and the clinicobiological features of CLL in relationship of these factors, is also provided.
authors.affiliation: Clinical and Experimental Onco-Hematology Unit, Centro di Riferimento Oncologico, IRCCS, Aviano (PN), Italy. micheledalbo@gmail.com
authors.forename: Michele
authors.initials: M
authors.lastname: Dal-Bo
authors.forename: Francesco
authors.initials: F
authors.lastname: Bertoni
authors.forename: Francesco
authors.initials: F
authors.lastname: Forconi
authors.forename: Antonella
authors.initials: A
authors.lastname: Zucchetto
authors.forename: Riccardo
authors.initials: R
authors.lastname: Bomben
authors.forename: Roberto
authors.initials: R
authors.lastname: Marasca
authors.forename: Silvia
authors.initials: S
authors.lastname: Deaglio
authors.forename: Luca
authors.initials: L
authors.lastname: Laurenti
authors.forename: Dimitar G
authors.initials: DG
authors.lastname: Efremov
authors.forename: Gianluca
authors.initials: G
authors.lastname: Gaidano
authors.forename: Giovanni
authors.initials: G
authors.lastname: Del Poeta
authors.forename: Valter
authors.initials: V
authors.lastname: Gattei
mesh_terms: D015703:Antigens, CD / Q000235:genetics / Q000276:immunology; D002882:Chromosomes, Human, Pair 13; D017353:Gene Deletion; D015973:Gene Expression Regulation, Leukemic; D006801:Humans; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology; D011379:Prognosis; D011947:Receptors, Antigen, B-Cell / Q000235:genetics / Q000378:metabolism; D051746:ZAP-70 Protein-Tyrosine Kinase / Q000235:genetics / Q000276:immunology
chemical_list: D015703:Antigens, CD; D011947:Receptors, Antigen, B-Cell; D051746:ZAP-70 Protein-Tyrosine Kinase; C497261:ZAP70 protein, human