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 [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

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

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

Reference

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

Reference

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

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

» Japanese version

All links

Disease (1)   
   OMIM (1)   
Drug (7)   
   KEGG DRUG (7)   
Gene (2)   
   KEGG ORTHOLOGY (1)   
   KEGG GENES (1)   
Literature (4)   
   PubMed (4)   
All databases (14)   

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