| H00006 | |
| H番号 | H00006 |
| 名称 | 有毛細胞白血病; ヘアリー細胞白血病; ヘアリーセル白血病 |
| 概要 | 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. |
| カテゴリ | がん |
| ネットワーク | - |
| 病因遺伝子 | p53 (mutation) [HSA:7157] [KO:K04451] Bcl-6 (mutation) [HSA:604] [KO:K15618] Cyclin D1 (overexpression) [HSA:595] [KO:K04503] |
| 病原体 | - |
| 環境要因 | - |
| 発癌物質 | - |
| 治療薬 | クラドリビン [DR:D01370] リツキシマブ [DR:D02994] (CD20陽性) インターフェロンアルファ (NAMALWA) [DR:D03305] |
| コメント | - |
| リンク | ICD-11: 2A82.2 ICD-10: C91.4 MeSH: D007943 |
| 文献 | PMID:12670461 著者 Zuzel M, Cawley JC. タイトル The biology of hairy cells. 雑誌 Best Pract Res Clin Haematol 16:1-13 (2003) DOI:10.1016/S1521-6926(02)00082-8 |