KEGG   DISEASE: Polycythemia vera
Entry
H00012                      Disease                                
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
Brite
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Neoplasms of haematopoietic or lymphoid tissues
   Myeloproliferative neoplasms
    2A20  Non mast cell myeloproliferative neoplasms
     H00012  Polycythemia vera
Pathway-based classification of diseases [BR:br08402]
 Cellular process
  nt06535  Efferocytosis
   H00012  Polycythemia vera
Network
nt06535 Efferocytosis
Gene
(PV) JAK2 [HSA:3717] [KO:K04447]
Drug
Ruxolitinib phosphate [DR:D09960]
Ropeginterferon alfa-2b [DR:D11027]
Sodium phosphate P 32 [DR:D05870]
Other DBs
ICD-11: 2A20.4
ICD-10: D45
MeSH: D011087
OMIM: 263300
Reference
  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
Reference
  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
Reference
  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
Reference
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
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