Search Database ICD-O-3 Code Lists

Name

Polycythemia vera (PV)

ICD-O-3 Morphology

9950/3: Polycythemia vera
Effective 2001 and later

Reportable

for cases diagnosed 2001 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421). Blood and bone marrow are the primary sites of involvement.

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Polycythemia Vera (PV) is part of the Myeloproliferative neoplasms (MPN) lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B2)

The primary therapy for PV includes the following, which is used to maintain the hematocrit below 45%. The goal of therapy for PV is maintenance.
* Phlebotomy
* Cytoreductive therapy

JAK2 status is collected in the following SSDI:
* NAACCR# 3862: JAK2

Diagnostic Confirmation

This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.

Module Rule

None

Alternate Names

Accelerated polycythemia vera
Blast phase polycythemia vera
Chronic erythremia [OBS]
Polycythemia rubra vera (PRV)
Post-polycythemic myelofibrosis (post-PV MF)
Primary polycythemia
Proliferative polycythemia
Spent phase polycythemia
Splenomegalic polycythemia
Vaquez-Osler's disease

Definition

"Polycythaemia vera (PV) is a myeloproliferative neoplasm (MPN) characterized by erythrocytosis, frequently accompanied by leukocytosis and/or thrombocytosis, and typically associated with activating JAK2 mutations." (WHO 5th edition).

Masked polycythemia has been proposed for patients that have a JAK2 mutation and suspicion of PV; however, per WHO 5th edition, these should be assigned to MPN, NOS (9975/3).

Clinical studies needed for diagnosis of PV include the detection of the JAK2p.V617F or functionally similar mutation (e.g. JAK2exon 12) and subnormal serum erythropoietin levels.

Post-polycythemia myelofibrosis (post-PV MF) is the same primary as polycythemia vera. It is usually associated with leukoerythroblasts findings, including poikilocytosis and teardrop-shaped red blood cells (dacrocytes).

Reactive or secondary polycythemias are not the same thing as polycythemia vera and are not reportable.

The WHO 5th edition Blue Book for Hematolymphoid Neoplasms has a staging system for PV called the Mutation-Enhanced International Prognostic Scoring System for Polycythemia Vera (MIPSS-PV). This staging system is not collected.

Definitive Diagnostic Methods

Clinical diagnosis
Cytogenetics
Genetic testing

Genetics Data

Janus kinase-2, JAK2
JAK2 exon 12
JAK2 V617F

Immunophenotyping

None

Treatments

Chemotherapy
Immunotherapy
Other therapy

Transformations to

Transformations from

None

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

D45 Polycythemia vera
C94.1 Chronic erythremia

Corresponding ICD-10-CM Codes (U.S. only)

D45 Polycythemia vera (effective October 01, 2015)

Signs and Symptoms

Budd-Chiari syndrome
Dizziness
Double vision or seeing dark or blind spots that come and go
Elevated white blood cell count
Fatigue
Feeling of pressure or fullness below the ribs on the left side
Headaches/migraines
Hypertension
Itching all over the body, especially after being in warm or hot water
Myocardial ischemia or stroke
Night sweats
Pruritis
Reddened face that looks like a blush or sunburn
Splenomegaly
Stroke
Vascular abnormalities
Weakness
Weight loss for no reason

Diagnostic Exams

Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunophenotyping
JAK 2 gene mutation test
Lumbar puncture
Molecular analysis
Peripheral blood smear
Physical exam and history

Progression and Transformation

Up to 20% transform to myelodysplasia or AML (usually cause of death)

Epidemiology and Mortality

Age: 60 years median age
Incidence: 1.57 per 100,000 in US
Sex: Slight male predominance
Survival: >10 years with treatment

Sources

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Myeloproliferative neoplasms
Pages: Part A: 40-43

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577

PDQ® Adult Treatment Editorial Board. PDQ Myeloproliferative Neoplasms Treatment. Bethesda, MD: National Cancer Institute. Updated <09/27/24>. Available at: https://www.cancer.gov/types/myeloproliferative/hp/myeloproliferative-neoplasms-treatment. Accessed <01/22/25>. [PMID: 26389291]
Section: Myeloproliferative Neoplasms Treatment (PDQ®)-Health Professional Version
Pages: https://www.cancer.gov/types/myeloproliferative/hp/myeloproliferative-neoplasms-treatment
Glossary