Search Database ICD-O-3 Code Lists

Name

Polycythemia vera (PV) (ICD-O-4: 99500/3)

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

Primary polycythemia is a condition in which there is an overproduction of blood cells due to a neoplastic process. Secondary polycythemia vera is an over production of red blood cells caused by a co-morbidity, in this case, volume depletion, and is not reportable.

See entry "polycythemia" for the other polycythemia terms that are not reportable.

Diagnostic Confirmation

This neoplasm can be diagnosed clinically (diagnostic confirmation codes 5, 6, 8) or histologically (diagnostic confirmation codes 1, 2, 4)

Module Rule

None

Alternate Names

Accelerated polycythemia vera (ICD-O-4: 99500/3)
Blast phase polycythemia vera (ICD-O-4: 99500/3)
Chronic erythremia [OBS] (ICD-O-4: 99500/3)
Polycythemia rubra vera (PRV) (ICD-O-4: 99500/3)
Post-polycythemic myelofibrosis (post-PV MF) (ICD-O-4: 99500/3)
Primary polycythemia (ICD-O-4: 99500/3)
Proliferative polycythemia (ICD-O-4: 99500/3)
Spent phase polycythemia (ICD-O-4: 99500/3)
Splenomegalic polycythemia (ICD-O-4: 99500/3)
Vaquez-Osler's disease (ICD-O-4: 99500/3)

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).
Note: Primary erythrocytosis is not the same thing as primary polycythemia, and is not reportable

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
Histologic confirmation

Genetics Data

JAK2 V617F

Immunophenotyping

No specific immunophenotyping profile

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