KEGG   DISEASE: Polycythemia veraHelp
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 [BR:br08402]
 Cancers
  Cancers of haematopoietic and lymphoid tissues
   H00012  Polycythemia vera
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
BRITE hierarchy
Gene
JAK2 (mutation) [HSA:3717] [KO:K04447]
Drug
Mechlorethamine hydrochloride [DR:D04872]
Ruxolitinib phosphate [DR:D09960]
Sodium phosphate P 32 [DR:D05870]
Comment
ICD-O: 9950/3, Tumor type: Polycythemia vera
Other DBs
ICD-11: 2A20.4
ICD-10: D45
MeSH: D011087
Reference
PMID:16879013 (gene, tumor type)
  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:16919893 (marker)
  Authors
Michiels JJ, Bernema Z, Van Bockstaele D, De Raeve H, Schroyens W.
  Title
Current diagnostic criteria for the chronic myeloproliferative disorders (MPD) essential thrombocythemia (ET), polycythemia vera (PV) and chronic idiopathic myelofibrosis (CIMF).
  Journal
Pathol Biol (Paris) 55:92-104 (2007)
DOI:10.1016/j.patbio.2006.06.002
Reference
PMID:15217712 (marker)
  Authors
Klippel S, Pahl HL.
  Title
Molecular markers for the diagnosis of Philadelphia chromosome negative myeloproliferative disorders.
  Journal
Pathol Biol (Paris) 52:267-74 (2004)
DOI:10.1016/j.patbio.2003.09.019
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