skip navigation links
National Cancer Institute
Enterprise Vocabulary Services
Box
NCIm Version: 201808 (Browser Version 2.9, using LexEVS 6.5.1)
SearchBox Top
SearchBox Bottom
Polycythemia Vera (CUI C0032463) Suggest changes to this concept
Add to Cart

Terms & Properties

Concept Unique Identifier (CUI): C0032463

NCI Thesaurus Code: C3336  (see NCI Thesaurus info)

Semantic Type: Neoplastic Process

NCIt Definition: A chronic myeloproliferative neoplasm characterized by an increased red blood cell production. The bone marrow is hypercellular due to a panmyelotic proliferation typically characterized by pleomorphic megakaryocytes. The major symptoms are related to hypertension, splenomegaly or to episodes of thrombosis and/or hemorrhage.

RADLEX Definition: A myeloproliferative disorder of unknown etiology, characterized by abnormal proliferation of all hematopoietic bone marrow elements and an absolute increase in red cell mass and total blood volume, associated frequently with splenomegaly, leukocytosis, and thrombocythemia. Hematopoiesis is also reactive in extramedullary sites (liver and spleen). In time myelofibrosis occurs. [MeSH]

GARD Definition: Polycythemia vera (PV) is a condition characterized by an increased number of red blood cells in the bloodstream. Affected people may also have excess white blood cells and platelets. These extra cells cause the blood to be thicker than normal, increasing the risk for blood clots that can block blood flow in arteries and veins. People with PV have an increased risk of deep vein thrombosis which can cause a pulmonary embolism, heart attack, and stroke. Most cases of PV are not inherited and are acquired during a person's lifetime. In rare cases, the risk for PV runs in families and may be inherited in an autosomal dominant manner. The condition has been associated with mutations in the JAK2 and TET2 genes. - this information is from GARD/ORDR/NCATS.

PDQ Definition: A chronic myeloproliferative disorder characterized by an increased red blood cell production. Excessive proliferation of the myeloid lineage is observed as well. The major symptoms are related to hypertension or to vascular abnormalities caused by the increased red cell mass. The cause is unknown. With currently available treatment, the median survival exceeds 10 years. (WHO, 2001) Check for active clinical trials using this agent. (NCI Thesaurus)

NCI-GLOSS Definition: A disease in which there are too many red blood cells in the bone marrow and blood, causing the blood to thicken. The number of white blood cells and platelets may also increase. The extra blood cells may collect in the spleen and cause it to become enlarged. They may also cause bleeding problems and make clots form in blood vessels.

CTEP Definition: CTEP_For_FDA: 90600192/Polycythemia vera

CSP Definition: myeloproliferative disorder characterized by abnormal proliferation of all hematopoietic bone marrow elements and an absolute increase in red cell mass and total blood volume; associated frequently with splenomegaly, leukocytosis, and thrombocythemia.

MSH Definition: A myeloproliferative disorder of unknown etiology, characterized by abnormal proliferation of all hematopoietic bone marrow elements and an absolute increase in red cell mass and total blood volume, associated frequently with splenomegaly, leukocytosis, and thrombocythemia. Hematopoiesis is also reactive in extramedullary sites (liver and spleen). In time myelofibrosis occurs.

Synonyms & Abbreviations: (see Synonym Details)
Acquired primary erythocytosis
Chronic erythraemia [obs]
Chronic erythremia [obs]
Disease, Osler-Vaquez
Erythremias
ERYTHREMIA
Osler Vaquez Disease
OSLER VAQUEZ DIS
Osler's disease
OSLER-VAQUEZ DISEASE
Osler-Vaquez syndrome
p.vera
Polycythaemia rubra vera
Polycythaemia vera (clinical)
Polycythaemia vera (D45)
Polycythaemia vera
Polycythemia Ruba Veras
Polycythemia Ruba Vera
Polycythemia Rubra Veras
Polycythemia rubra vera
Polycythemia vera (clinical)
Polycythemia vera (disorder)
Polycythemia vera (morphologic abnormality)
Polycythemia Vera [Disease/Finding]
polycythemia vera
POLYCYTHEMIA, PRIMARY
POLYCYTHEMIA, SPLENOMEGALIC
Polycythemias, Primary
Polyzythaemia vera
PPP - Primary proliferative polycythaemia
PPP - Primary proliferative polycythemia
Primary polycythaemia
Primary Polycythemias
Primary polycythemia
Primary proliferative polycythaemia
Primary proliferative polycythemia
Proliferative polycythaemia
Proliferative polycythemia
PRV - Polycythaemia rubra vera
PRV - Polycythemia rubra vera
PRV
PV
Ruba Vera, Polycythemia
Ruba Veras, Polycythemia
Vaquez disease
Vaquez's disease
VAQUEZ-OSLER DISEASE
Vera, Polycythemia Ruba
Vera, Polycythemia Rubra
Veras, Polycythemia Ruba
Veras, Polycythemia Rubra

External Source Codes: 
NCI Thesaurus Code C3336 (see NCI Thesaurus info)

Other Properties: Property Definitions
Name Value Source
ACTIVE 1 SNOMEDCT_US
AQL BL BS CF CH CI CL CN CO DG DH DI DT EC EH EM EN EP ET GE HI IM ME MI MO NU PA PC PP PS PX RH RT SC SE SU TH UL UR VE VI MSH
CASE_SIGNIFICANCE_ID 900000000000017005 SNOMEDCT_US
CASE_SIGNIFICANCE_ID 900000000000448009 SNOMEDCT_US
CHARACTERISTIC_TYPE_ID 900000000000011006 SNOMEDCT_US
Contributing_Source CTEP NCI
Contributing_Source CTRP NCI
Contributing_Source NICHD NCI
CTV3ID B934. SNOMEDCT_US
CTV3ID XU7HM SNOMEDCT_US
DATE_CREATED 2013-01-09T00:04:00 GARD
DATE_CREATED 2014-01-17T00:03:00 GARD
DATE_CREATED 2015-01-22T00:04:00 GARD
DATE_LAST_MODIFIED 2011-06-03 PDQ
DATE_LAST_MODIFIED 2013-01-09T00:04:00 GARD
DATE_LAST_MODIFIED 2014-01-17T00:03:00 GARD
DC 1 MSH
DEFINITION_STATUS_ID 900000000000073002 SNOMEDCT_US
DEFINITION_STATUS_ID 900000000000074008 SNOMEDCT_US
DID 2004-0979 CSP
DISEASE_IDENTIFIER_ID 23242 GARD
DISEASE_IDENTIFIER_ID 33716 GARD
DISEASE_IDENTIFIER_ID 33717 GARD
DISEASE_IDENTIFIER_ID 33718 GARD
DISEASE_IDENTIFIER_ID 33719 GARD
DISEASE_IDENTIFIER_ID 5449 GARD
DISEASE_IDENTIFIER_ID 5450 GARD
DISEASE_IDENTIFIER_ID 5451 GARD
DX 19660101 MSH
EFFECTIVE_TIME 20020131 SNOMEDCT_US
EXCLUDES1 familial polycythemia (D75.0) ICD10CM
EXCLUDES1 secondary polycythemia (D75.1) ICD10CM
GENELOCUS ,9,p,2,4, OMIM
GENESYMBOL JAK2 OMIM
GENESYMBOL THCYT3 OMIM
HAS_GARD_PAGE true GARD
IAN DEFAULT ICD10
ICD-O-3_Code 9950/3 NCI
ICD-O-3_CODE 9950/3 SNOMEDCT_US
IDENTIFIER_SOURCE NHB GARD
IDENTIFIER_SOURCE Orp GARD
IDENTIFIER_TYPE_ID 1 GARD
IS_ACTIVE true GARD
IS_RARE true GARD
IS_SPANISH false GARD
LAST_REVIEW_ACTION Modified GARD
LAST_REVIEW_DATE 2009-01-23T00:00:00 GARD
MDA 19990101 MSH
MESH_DEFINITION A myeloproliferative disorder of unknown etiology, characterized by abnormal proliferation of all hematopoietic bone marrow elements and an absolute increase in red cell mass and total blood volume, associated frequently with splenomegaly, leukocytosis, and thrombocythemia. Hematopoiesis is also reactive in extramedullary sites (liver and spleen). In time myelofibrosis occurs. NDFRT
MESH_DUI D011087 NDFRT
MESH_NAME Polycythemia Vera NDFRT
MESH_UI M0017168 NDFRT
MIMTYPE 3 OMIM
MIMTYPEMEANING Phenotype description, molecular basis known. OMIM
MIMTYPEVALUE pound OMIM
MMR 20160701 MSH
MN C04.588.448.200.500 MSH
MN C15.378.190.250.500 MSH
MN C15.378.190.636.753 MSH
MN C15.378.400.200.500 MSH
MODIFIER_ID 900000000000451002 SNOMEDCT_US
NCI_THESAURUS_CODE C3336 PDQ
NDFRT_KIND DISEASE_KIND NDFRT
Neoplastic_Status Malignant NCI
NICHD_Hierarchy_Term Polycythemia Vera NCI
NUI N0000002454 NDFRT
ORDER_NO 03277 ICD10CM
ORIG_STY Cancer stage PDQ
PID 1986 PDQ
PRIMARY_PATH 10036057$10028578$10018865$10029104$Polycythaemia vera$Myeloproliferative disorders (excl leukaemias)$Haematopoietic neoplasms (excl leukaemias and lymphomas)$Neoplasms benign, malignant and unspecified (incl cysts and polyps) MDR
PRIMARY_SOC 10029104 MDR
PT_IN_VERSION 10.0 MDR
PT_IN_VERSION 10.1 MDR
PT_IN_VERSION 11.0 MDR
PT_IN_VERSION 11.1 MDR
PT_IN_VERSION 12.0 MDR
PT_IN_VERSION 12.1 MDR
PT_IN_VERSION 13.0 MDR
PT_IN_VERSION 13.1 MDR
PT_IN_VERSION 14.0 MDR
PT_IN_VERSION 14.1 MDR
PT_IN_VERSION 15.0 MDR
PT_IN_VERSION 15.1 MDR
PT_IN_VERSION 16.0 MDR
PT_IN_VERSION 16.1 MDR
PT_IN_VERSION 17.0 MDR
PT_IN_VERSION 17.1 MDR
PT_IN_VERSION 18.0 MDR
PT_IN_VERSION 18.1 MDR
PT_IN_VERSION 19.0 MDR
PT_IN_VERSION 19.1 MDR
PT_IN_VERSION 20.0 MDR
PT_IN_VERSION 20.1 MDR
PT_IN_VERSION 8.0 MDR
PT_IN_VERSION 8.1 MDR
PT_IN_VERSION 9.0 MDR
PT_IN_VERSION 9.1 MDR
RARE_DISEASE_URL http://rarediseases.info.nih.gov/gard/7422/polycythemia-vera/resources/1 GARD
RXAUI 3155315 RXNORM
RXAUI 3210857 RXNORM
RXAUI 3222043 RXNORM
RXAUI 3222044 RXNORM
RXAUI 3606237 RXNORM
RXAUI 5932044 RXNORM
RXAUI 5932051 RXNORM
RXCUI 1023815 RXNORM
SMQ_TERM_ADDVERSION 10.0 MDR
SMQ_TERM_CAT A MDR
SMQ_TERM_LEVEL 4 MDR
SMQ_TERM_LEVEL 5 MDR
SMQ_TERM_LMVERSION 14.0 MDR
SMQ_TERM_SCOPE 2 MDR
SMQ_TERM_STATUS A MDR
SMQ_TERM_WEIGHT 0 MDR
SNOMED_CID 109992005 NDFRT
SUBSET_MEMBER 446608001~MAPTARGET~9950/3 SNOMEDCT_US
SUBSET_MEMBER 447562003~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPADVICE~ALWAYS D45 | POSSIBLE REQUIREMENT FOR MORPHOLOGY CODE SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPTARGET~D45 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~ALWAYS D45 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF FAMILIAL POLYCYTHEMIA VERA CHOOSE D75.0 | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447639009 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~2 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~IFA 127066000 | Familial polycythemia vera | SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~OTHERWISE TRUE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~D45 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~D75.0 SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~B934. SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~XU7HM SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
TERM_LEVEL 4 ICDO
TERM_LEVEL Synonym ICDO
TERMUI T032600 MSH
TERMUI T032601 MSH
TERMUI T032602 MSH
TERMUI T768257 MSH
TERMUI T842296 MSH
TERMUI T842297 MSH
TH GHR (2014) MSH
TH NLM (1966) MSH
TH NLM (1995) MSH
TH NLM (2009) MSH
TH NLM (2014) MSH
TH OMIM (2013) MSH
TH ORD (2010) MSH
TH UNK (19XX) MSH
TYPE_ID 900000000000003001 SNOMEDCT_US
TYPE_ID 900000000000013009 SNOMEDCT_US

Additional Concept Data:  (none)

URL to Bookmark: https://ncim-stage.nci.nih.gov/ncimbrowser/ConceptReport.jsp?dictionary=NCI Metathesaurus&code=C0032463

Mainbox Bottom