| H00006 | |
| H number | H00006 |
| Name | Hairy cell leukemia |
| Description | Hairy cell Leukemia (HCL) is a chronic lymphoproliferative disorder that is defined, according to the WHO classification, as a mature (peripheral) B-cell neoplasm. HCL accounts for between 2-3% of all leukemia cases, with about 600 new cases diagnosed in the U.S. each year. With regard to genetic abnormalities of potential pathogenetic importance in HCL, only a few have been described. Mutations of p53 and Bcl-6 have been reported in about one-third of cases, but their functional significance is unclear. Over-expression of cyclin D1, an important cell cycle regulator, has been reported in cases of HCL as well as mantle cell lymphoma (MCL) and in a few cases of chronic lymphocytic leukemia among lymphoid malignancies. |
| Category | Cancer |
| Network | - |
| Gene | p53 (mutation) [HSA:7157] [KO:K04451] Bcl-6 (mutation) [HSA:604] [KO:K15618] Cyclin D1 (overexpression) [HSA:595] [KO:K04503] |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| Drug | Cladribine [DR:D01370] Moxetumomab pasudotox [DR:D09932] Pentostatin [DR:D00155] Interferon alfa-2b [DR:D02745] |
| Comment | - |
| Other DBs | ICD-11: 2A82.2 ICD-10: C91.4 MeSH: D007943 |
| Reference | PMID:12670461 AUTHORS Zuzel M, Cawley JC. TITLE The biology of hairy cells. JOURNAL Best Pract Res Clin Haematol 16:1-13 (2003) DOI:10.1016/S1521-6926(02)00082-8 |