Polycythemia vera

Name
Polycythemia vera
ICD-O-3 Morphology
9950/3
Effective 2001 and later
Reportable
for cases diagnosed 2001 and later
Primary Site(s)
C421

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Grade
9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
None
Alternate Names
Chronic erythremia [OBS]
Cryptogenic polycythemia
Erythremia
Erythrocytosis megalosplenica
Myelopathic polycythemia
Osler-Vaquez disease
Plethora vera
Polycythemia rubra vera
Polycythemia with chronic cyanosis
Primary polycythemia
Proliferative polycythemia
PRV
PV
Spent phase polycythemia
Splenomegalic polycythemia
Vaquez-Osler's disease
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. 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 fequency). 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. The patient usually presents with hypertension or vascular abnormalities; nearly 20% have an episode of DVT, myocardial ischemia, or a stroke. The patient may present with an elevated platelet count, elevated hemoglobin/hematocrit, elevated white cell count, or enlarged spleen. Treatment does not cure PV but does control it. 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.
Definitive Diagnostic Methods
Bone marrow biopsy
Clinical diagnosis
JAK2
Genetics Data
Janus kinase-2, JAK2
Immunophenotyping
No immunophenotype features have been reported
Treatments
Blood thinners, anti-coagulant medications, sometimes aspirin
Chemotherapy
Phlebotomy
Transformations from
No Transformations
Corresponding ICD-9 Codes
238.4 Polycythemia vera
Corresponding ICD-10 Codes
D45 Polycythemia vera
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
D45 Polycythemia vera
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None