The biology of hairy-cell leukaemia.
OBJECTIVE
The biology of hairy-cell leukaemia is reviewed, focussing first on the hairy cell itself and then on its interactions with the microenvironment.


RESULTS
Hairy cells are highly activated clonal B cells related to memory cells, normally resident in the marginal zone of the spleen. Their activation results from multiple stimuli arising from the microenvironment, autocrine cytokines and the still unknown transforming oncogenic event(s) responsible for the disease. Protein kinase Cepsilon is a central player in the activation process.


CONCLUSIONS
The activation of hairy cells makes them unusually sensitive to interferon and nucleosides. Future important research topics include characterization of the oncogenic events responsible for the disease and for its associated differentiation block.
Cawley
John C
JC
Division of Haematology, School of Cancer Studies, University of Liverpool, Liverpool, UK. haem@liv.ac.uk
Hawkins
Stephen F
SF
D001402:B-Lymphocytes / Q000378:metabolism / Q000473:pathology*; D016207:Cytokines / Q000378:metabolism; D048049:Extracellular Signal-Regulated MAP Kinases / Q000378:metabolism; D006801:Humans; D007943:Leukemia, Hairy Cell / Q000378:metabolism / Q000473:pathology*; D008213:Lymphocyte Activation; D011493:Protein Kinase C / Q000378:metabolism; D018121:Receptors, Cytokine / Q000378:metabolism
D016207:Cytokines; D018121:Receptors, Cytokine; D011493:Protein Kinase C; D048049:Extracellular Signal-Regulated MAP Kinases