The pathophysiology of the hairy cell.
HCs are clonal late B cells that are related to memory cells and display specific features of activation. Many of the distinctive features of HCs (eg, morphology, TRAP) are related to this specific activation. Many of the distinctive histologic features of HCL can be related to constitutive production of cytokines (eg, FGF, fibrosis) and to the expression/activation of adhesion receptors (eg, alpha(4)beta(1), alpha(5)beta(1) and alpha(v)beta(3) integrins, CD44v3). HCs usually have mutated IGVH genes and have no consistent or specific chromosome abnormalities (5q additions and 7q deletions in a minority). The signals that are responsible for several of the phenotypic features of HCs have been identified, but the nature of the underlying oncogenic events remains unknown.
Cawley
J C
JC
Department of Haematology, University of Liverpool, Third Floor Duncan Building, Daulby Street, Liverpool L69 3GA, UK. haem@liverpool.ac.uk
D001402:B-Lymphocytes / Q000276:immunology* / Q000473:pathology; D002448:Cell Adhesion / Q000276:immunology; D002465:Cell Movement / Q000276:immunology*; D016207:Cytokines / Q000276:immunology; D020869:Gene Expression Profiling / Q000379:methods; D015973:Gene Expression Regulation, Leukemic / Q000276:immunology*; D049673:History, 20th Century; D049674:History, 21st Century; D006801:Humans; D007943:Leukemia, Hairy Cell / Q000266:history / Q000276:immunology* / Q000473:pathology* / Q000503:physiopathology / Q000628:therapy; D008213:Lymphocyte Activation / Q000276:immunology*; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology
D016207:Cytokines