title: Hairy cell leukaemia: biological and clinical overview from immunogenetic insights.
abstract: Hairy cell Leukaemia (HCL) is a rare neoplasm of peripheral B cells which represents a paradox in oncology. Despite its largely unknown origin and behaviour, HCL is one of the few example of dramatic success in the treatment of a malignancy. The recent steps forward to understanding the biology of HCL from immunogenetic and genomic studies have recently provided new insight into diagnosis and prognosis. Several data from immunoglobulin gene (IG) analysis have provided hints regarding the cell of origin and the ongoing selective interactions of the tumour BCR with environmental stimuli. It has also recently emerged that an unmutated status of the HCL IG can be associated with failure to respond to cladribine, genetic abnormalities indicative of poor outcome and aggressive disease. These observations suggest a central role of the tumour B-cell receptor in defining the outcome of HCL and that that IG gene analysis may have biological and prognostic relevance. Hopefully, IG analysis will help tailor treatment strategies for the most aggressive cases.
authors.affiliation: Ematologia e Trapianti, Università di Siena & AOUS, Siena, Italy. forconif@unisi.it
authors.forename: Francesco
authors.initials: F
authors.lastname: Forconi
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001402:B-Lymphocytes / Q000187:drug effects* / Q000276:immunology / Q000378:metabolism; D017338:Cladribine / Q000008:administration & dosage; D006801:Humans; D055261:Immunogenetic Phenomena / Q000187:drug effects*; D007143:Immunoglobulin Heavy Chains / Q000235:genetics; D007943:Leukemia, Hairy Cell / Q000188:drug therapy* / Q000235:genetics / Q000276:immunology; D009154:Mutation; D011947:Receptors, Antigen, B-Cell / Q000235:genetics; D000069283:Rituximab
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D007143:Immunoglobulin Heavy Chains; D011947:Receptors, Antigen, B-Cell; D017338:Cladribine; D000069283:Rituximab