Hairy cell phenotypes

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Phenotypic changes of phytohaemagglutinin-stimulated hairy cells

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The phenotype of the neoplastic cells of hairy cell leukemia studied with monoclonal antibodies.

Theobjective of reviewing Hairy Cell Leukaemia may be achieved by emphasising the condition as a category of chronic lymphocytic leukaemia with hair like protrusions of the cytoplasm situated on the aberrant B cell surface. An enlarged spleen may be discerned along with pancytopenia as a presenting symptom. Reticulin stains exhibit broad, dense reticulum fibres surrounding the individual or aggregates of leukaemia cells with fibrotic extensions into the abutting bone marrow. Competing interests The authors have declared that no competing interests exist. Originally delineated in by Bouroncle and colleagues, hairy cell leukaemia HCL is a category of chronic lymphocytic leukaemia with the terminology based on the hair like protrusions of cytoplasm situated on the surface of aberrant B cell 1. The leukaemia demonstrates a male predominance M:F 2.
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The phenotype of the neoplastic cells of hairy cell leukemia studied with monoclonal antibodies.

Hairy cell leukemia HCL and hairy cell leukemia-variant HCL-v are rare diseases with overlapping clinico-pathological features. Most patients respond to apoptosis-inducing purine analogs with long-term complete remission [ 3 — 6 ] and refractory patients respond well to immunotoxin therapy [ 7 — 9 ], resulting in a near normal life expectancy [ 10 ]. The neoplastic cells also have circumferential cytoplasmic projections but a prominent nucleolus and basophilic cytoplasm are common. Although the patients typically present at an older age with higher numbers of circulating hairy cells than observed in HCL, there is overlap in the spectrum of clinical features [ 1 , 11 , 13 ]; Furthermore the prominent nucleoli characteristic of HCL-v may be absent [ 14 ]. HCL-v is a more aggressive disease, with significantly shorter median survival and poor response to purine analogs [ 1 , 5 ]; however, complete remission has been achieved with rituximab and BL22 [ 8 , 15 ].
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This disease is rare, with fewer than 1 in 10, people being diagnosed with HCL during their lives. Although most patients are white males over the age of 50, it has been diagnosed in at least one teenager. Men are four to five times more likely to develop hairy cell leukemia than women. It does not appear to be hereditary, although occasional familial cases have been reported, usually showing a common HLA type.
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