title: Insight into the behavior of hairy cell leukemia by immunogenetic analysis.
abstract: The B cell receptor (BCR) is the functional distinguishing unit that defines any B cell. Immunoglobulin gene (IG) status is preserved in the neoplastic B cell clone and can provide an indicator of the maturation stage reached by the B cell prior to transformation. In hairy cell leukemia (HCL), several data from IG analysis provide clear hints regarding the cell of origin and the ongoing selective interactions of the tumor BCR with environmental stimuli: HCLs (i) have variable levels of IG somatic mutations, (ii) continue somatic mutations at low levels, (iii) have active processes of IG class switching after transformation, and (iv) have a relatively high frequency of selective events in the light chain of the BCR. It has recently emerged that an unmutated status of the HCL-derived IG can be associated with failure to respond to cladribine, genetic abnormalities indicative of poor outcome, and aggressive disease. These observations suggest that IG gene analysis may have biological and prognostic relevance in HCL and merits further characterization.
authors.affiliation: Sezione di Ematologia e Trapianti, Dipartimento di Medicina Clinica e Scienze Immunologiche, Università di Siena & AOUS, Siena, Italy. forconif@unisi.it
authors.forename: Francesco
authors.initials: F
authors.lastname: Forconi
authors.forename: Emanuele
authors.initials: E
authors.lastname: Cencini
authors.forename: Elisa
authors.initials: E
authors.lastname: Sozzi
authors.forename: Anna
authors.initials: A
authors.lastname: Sicuranza
authors.forename: Donatella
authors.initials: D
authors.lastname: Raspadori
authors.forename: Francesco
authors.initials: F
authors.lastname: Lauria
mesh_terms: D017338:Cladribine / Q000627:therapeutic use; D005803:Genes, Immunoglobulin; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics; D007943:Leukemia, Hairy Cell / Q000175:diagnosis / Q000235:genetics / Q000276:immunology*; D011379:Prognosis; D011947:Receptors, Antigen, B-Cell / Q000235:genetics*
chemical_list: D007143:Immunoglobulin Heavy Chains; D011947:Receptors, Antigen, B-Cell; D017338:Cladribine