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Definition of Hairy cell leukemia
Hairy cell leukemia definition - medical term
A form of chronic leukemia in which malignant B-lymphocytes
(a type of white blood cell) are seen in the bone marrow, spleen, and peripheral
blood and when viewed under the microscope, these cells appear to be covered with
tiny hair-like projections. Hairy cell leukemia represents 2% of all leukemia.
The characteristic features of the disease include marked enlargement of the
spleen (splenomegaly), low blood cell counts (pancytopenia), a relatively small
number of circulating tumor cells with a hairy appearance, and infiltration of the
spleen, liver, and bone marrow by the leukemic hairy cells.
Annexin A1 (ANXA1) is a gene that is unregulated in hairy cell leukemia. ANXA1
protein expression is specific to hairy cell leukemia. Detection of ANXA1 (by immunocytochemical
means) provides a simple, highly sensitive and specific assay for the diagnosis
of hairy cell leukemia.
Splenectomy (surgical removal of the spleen) provides palliation (some help but
not a cure). Treatment is usually with drugs, principally interferon alfa and purine
analogues such as cladribine and pentostatin. For resistant cases, a promising immunotoxin
has been developed that targets CD22, a molecule expressed exclusively on the surface
of B-cells, including virtually all hairy cells.
Hairy cell leukemia was first described in 1958 and was misleadingly called leukemic
reticuloendotheliosis.
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