Polycythemia vera

Polycythemia vera
ICD-O-3 Morphology
9950/3: Polycythemia vera
Effective 2001 and later
for cases diagnosed 2001 and later
Primary Site(s)
Primary site must be bone marrow (C421)

Not Applicable
Module Rule
Alternate Names
Acquired true primary polycythemia
Chronic erythremia [OBS]
Cryptogenic polycythemia
Erythrocytosis megalosplenica
Myelopathic polycythemia
Osler-Vaquez disease
Plethora vera
Polycythemia rubra vera
Polycythemia vera, acquired
Polycythemia with chronic cyanosis
Primary polycythemia
Proliferative polycythemia
Spent phase polycythemia
Splenomegalic polycythemia
Vaquez-Osler's disease
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.

PV arises in clonal hematopoietic stem cell, characterized by increased RBC production independent of mechanisms that normally regulate erythropoiesis.
Abstractor Notes
The JAK2 mutation occurs in >96% of patients with PV, however JAK2 can also occur in other types of myeloprolific neoplasms (with less frequency).

The blood and bone marrow are the primary sites of involvement. The physician also correlates the morphologic findings in BM biopsy with other clinical and laboratory findings to firmly establish the diagnosis.

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.

There are three phases of Polycythemia Vera:
1. Prodromal, pre-polycythemia phase
2. Overt polycythemia phase
3. Spent or post-polycythemia myelofibrosis phase

The "spent phase" of polycythemia vera is the finding of myelofibrosis in the bone marrow. The myelofibrosis is a result of the PV. This is still the same primary, polycythemia vera, with the bone marrow showing the effect of the disease. This form of myelofibrosis is different from primary myelofibrosis, which is code 9961/3.

The diagnosis of "polycythemia" by itself is not reportable. To be reportable the diagnosis must be polycythemia vera, or some of the other alternate names listed above in the alternate names section.

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).
Definitive Diagnostic Methods
Bone marrow biopsy
Clinical diagnosis
Genetic testing
Genetics Data
Janus kinase-2, JAK2
Transformations from
Corresponding ICD-9 Codes
238.4 Polycythemia vera
Corresponding ICD-10 Codes
D45 Polycythemia vera
Corresponding ICD-10-CM Codes (U.S. only)
D45 Polycythemia vera (effective October 01, 2015)
Signs and Symptoms
Budd-Chiari syndrome
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
Itching all over the body, especially after being in warm or hot water
Reddened face that looks like a blush or sunburn
Weight loss for no reason
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