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Name
Polycythemia vera
ICD-O-1 Morphology
9842/3: Chronic erythremia
9950/1: Polycythemia vera
ICD-O-2 Morphology
9842/3: Chronic erythremia
9950/1: Polycythemia vera
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
Treatment is used for control, not cure. The patient has phlebotomy (removal of blood, usually a pint every other day) until the hematocrit reaches a normal level. Then blood is removed every few months as needed.
Aspirin was previously documented as treatment for MPN, NOS. This was found to be incorrect. Treatment has been updated based on the NCI website. Aspirin is given to patients with PV to reduce bone pain. The aspirin is not used to manage the cancer. Treatment has been updated based on the NCI website (updated 6/12/15).
Aspirin was previously documented as treatment for MPN, NOS. This was found to be incorrect. Treatment has been updated based on the NCI website. Aspirin is given to patients with PV to reduce bone pain. The aspirin is not used to manage the cancer. Treatment has been updated based on the NCI website (updated 6/12/15).
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.
Grade
Not Applicable
Module Rule
None
Alternate Names
Acquired true primary polycythemia
Chronic erythremia [OBS]
Cryptogenic polycythemia
Erythremia
Erythrocytosis megalosplenica
Masked polycythemia
Myelopathic polycythemia
Osler-Vaquez disease
Plethora vera
Polycythemia rubra vera
Polycythemia vera, acquired
Post-polycythemic myelofibrosis
Post-PV MF
Primary polycythemia
Proliferative polycythemia
PRV
PV
Spent phase polycythemia
Splenomegalic polycythemia
Vaquez-Osler's disease
Definition
Polycythemia vera (PV) is a chronic myeloproliferative neoplasm (MPN) characterized by increased red blood cell (RBC) production independent of the mechanisms that normally regular erythropoiesis.
Virtually all patients carry the somatic JAK2 V617F gain-of-function mutation or another functionally similar JAK2 mutation that results in proliferation only only of the erythroid lineage but also of granulocytes and megakaryocytes.
Generally two phases of PV are recognized.
1. A polycythemic phase, associated with elevated hemoglobin level, elevated hematocrit, and increased RBC mass
2. A spent phase or post-polycythemic myelofibrosis phase (post-PV myelofibrosis), in which cytopenias, including anemia, are associated with ineffective hematopoiesis, bone marrow fibrosis, extramedullary hematopoiesis, and hypersplenism.
The natural progression of PV also includes a low incidence of evolution to a myelodysplastic/pre-leukemic phases and/or blast phase.
The diagnosis requires integration of clinical, laboratory, and bone marrow histological features. Diagnosis of PV requires meeting either all 3 major criteria, or the first 2 major criteria and the minor criterion.
Major criteria
1. Hemoglobin .16.5 g/dL in men, Hemoglobin .16.0 g/dL in women or, Hematocrit .49% in men, Hematocrit .48% in women or, increased red cell mass (RCM)
2. BM biopsy showing hypercellularity for age with trilineage growth (panmyelosis) including prominent erythroid, granulocytic, and megakaryocytic proliferation with pleomorphic, mature megakaryocytes (differences in size)
3. Presence of JAK2V617F or JAK2 exon 12 mutation
Minor criterion
Subnormal serum erythropoietin level
Virtually all patients carry the somatic JAK2 V617F gain-of-function mutation or another functionally similar JAK2 mutation that results in proliferation only only of the erythroid lineage but also of granulocytes and megakaryocytes.
Generally two phases of PV are recognized.
1. A polycythemic phase, associated with elevated hemoglobin level, elevated hematocrit, and increased RBC mass
2. A spent phase or post-polycythemic myelofibrosis phase (post-PV myelofibrosis), in which cytopenias, including anemia, are associated with ineffective hematopoiesis, bone marrow fibrosis, extramedullary hematopoiesis, and hypersplenism.
The natural progression of PV also includes a low incidence of evolution to a myelodysplastic/pre-leukemic phases and/or blast phase.
The diagnosis requires integration of clinical, laboratory, and bone marrow histological features. Diagnosis of PV requires meeting either all 3 major criteria, or the first 2 major criteria and the minor criterion.
Major criteria
1. Hemoglobin .16.5 g/dL in men, Hemoglobin .16.0 g/dL in women or, Hematocrit .49% in men, Hematocrit .48% in women or, increased red cell mass (RCM)
2. BM biopsy showing hypercellularity for age with trilineage growth (panmyelosis) including prominent erythroid, granulocytic, and megakaryocytic proliferation with pleomorphic, mature megakaryocytes (differences in size)
3. Presence of JAK2V617F or JAK2 exon 12 mutation
Minor criterion
Subnormal serum erythropoietin level
Definitive Diagnostic Methods
Bone marrow biopsy
Clinical diagnosis
Genetic testing
Genetics Data
Immunophenotyping
None
Treatments
Chemotherapy
Immunotherapy
Other therapy
Transformations to
Transformations from
None
Same Primaries
Corresponding ICD-9 Codes
238.4 Polycythemia vera
207.1 Chronic erythremia
Corresponding ICD-10 Codes
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 hemoglobin, hematocrit, or platelet count
Elevated white blood cell count
Feeling of pressure or fullness below the ribs on the left side
Headaches
Hypertension
Itching all over the body, especially after being in warm or hot water
Reddened face that looks like a blush or sunburn
Splenomegaly
Stroke
Vascular abnormalities
Weakness
Weight loss for no reason
Diagnostic Exams
Progression and Transformation
Up to 20% transform to myelodysplasia or AML (usually cause of death)
Epidemiology and Mortality
Age: 60 years median age
Incidence: 0.7 to 2.6 per 100,000 in US
Sex: Slight male predominance
Survival: >10 years with treatment
Sources
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Myeloproliferative neoplasms
Pages: 39-43
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Myeloproliferative neoplasms
Pages: 39-43
International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 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
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
National Cancer Institute
Section: General Information About Myeloproliferative Neoplasms
Pages: https://www.cancer.gov/types/myeloproliferative/hp/mds-mpd-treatment-pdq
Section: General Information About Myeloproliferative Neoplasms
Pages: https://www.cancer.gov/types/myeloproliferative/hp/mds-mpd-treatment-pdq
