H00012 | |
H number | H00012 |
Name | Polycythemia vera |
Description | 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. |
Category | Cancer |
Network | - |
Gene | (PV) JAK2 [HSA:3717] [KO:K04447] |
Pathogen | - |
Env factor | - |
Carcinogen | - |
Drug | Ruxolitinib phosphate [DR:D09960] Ropeginterferon alfa-2b [DR:D11027] Sodium phosphate P 32 [DR:D05870] |
Comment | - |
Other DBs | ICD-11: 2A20.4 ICD-10: D45 MeSH: D011087 OMIM: 263300 |
Reference | PMID:16879013 AUTHORS Cao M, Olsen RJ, Zu Y. TITLE Polycythemia vera: new clinicopathologic perspectives. JOURNAL Arch Pathol Lab Med 130:1126-32 (2006) DOI:10.1043/1543-2165(2006)130[1126:PV]2.0.CO;2 PMID:16781479 AUTHORS Chen G, Prchal JT. TITLE Polycythemia vera and its molecular basis: an update. JOURNAL Best Pract Res Clin Haematol 19:387-97 (2006) DOI:10.1016/j.beha.2005.07.003 PMID:17131059 AUTHORS Delhommeau F, Pisani DF, James C, Casadevall N, Constantinescu S, Vainchenker W. TITLE Oncogenic mechanisms in myeloproliferative disorders. JOURNAL Cell Mol Life Sci 63:2939-53 (2006) DOI:10.1007/s00018-006-6272-7 PMID:15781101 (JAK2) AUTHORS 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 TITLE Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders. JOURNAL Lancet 365:1054-61 (2005) DOI:10.1016/S0140-6736(05)71142-9 |