| H00012 | |
| H番号 | H00012 |
| 名称 | 真性赤血球増加症 |
| 概要 | Polycythemia vera (PV) is a clonal myeloproliferative disease characterized by an erythroid dominant trilineage proliferation of hematopoietic precursor cells. PV belongs to the family of chronic myeloproliferative disorders (MPD), which includes hematological diseases that share clinical and biological similarities, such as a hematopoietic stem cell origin: PV, essential thrombocythemia (ET), primary myelofibrosis (PMF), chronic myeloid leukemia (CML), some types of hypereosinophilic syndrome (HES), systemic mast cell disease (SMD) and other rare disorders. Recently, a specific point mutation in the Janus kinase 2 (JAK2) gene was described in a majority of PV patients and thus constitutes a sensitive diagnostic marker for the disease. |
| カテゴリ | がん |
| ネットワーク | - |
| 病因遺伝子 | (PV) JAK2 [HSA:3717] [KO:K04447] |
| 病原体 | - |
| 環境要因 | - |
| 発癌物質 | - |
| 治療薬 | シクロホスファミド水和物 [DR:D00287] ブスルファン [DR:D00248] ラニムスチン [DR:D01760] ヒドロキシカルバミド [DR:D00341] ルキソリチニブリン酸塩 [DR:D09960] ロペグインターフェロンアルファ-2b [DR:D11027] |
| コメント | - |
| リンク | ICD-11: 2A20.4 ICD-10: D45 MeSH: D011087 OMIM: 263300 |
| 文献 | PMID:16879013 著者 Cao M, Olsen RJ, Zu Y. タイトル Polycythemia vera: new clinicopathologic perspectives. 雑誌 Arch Pathol Lab Med 130:1126-32 (2006) DOI:10.1043/1543-2165(2006)130[1126:PV]2.0.CO;2 PMID:16781479 著者 Chen G, Prchal JT. タイトル Polycythemia vera and its molecular basis: an update. 雑誌 Best Pract Res Clin Haematol 19:387-97 (2006) DOI:10.1016/j.beha.2005.07.003 PMID:17131059 著者 Delhommeau F, Pisani DF, James C, Casadevall N, Constantinescu S, Vainchenker W. タイトル Oncogenic mechanisms in myeloproliferative disorders. 雑誌 Cell Mol Life Sci 63:2939-53 (2006) DOI:10.1007/s00018-006-6272-7 PMID:15781101 (JAK2) 著者 Baxter EJ, Scott LM, Campbell PJ, East C, Fourouclas N, Swanton S, Vassiliou GS, Bench AJ, Boyd EM, Curtin N, Scott MA, Erber WN, Green AR タイトル Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders. 雑誌 Lancet 365:1054-61 (2005) DOI:10.1016/S0140-6736(05)71142-9 |