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
Primary site must be bone marrow (C421)

Help me code for diagnosis year :

Grade
9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
None
Alternate Names
Chronic erythremia [OBS]
Cryptogenic polycythemia
Erythremia
Erythrocytosis megalosplenica
Masked polycythemia
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 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.
Definitive Diagnostic Methods
Bone marrow biopsy
Clinical diagnosis
Genetic testing
Genetics Data
Janus kinase-2, JAK2
Immunophenotyping
None
Treatments
Blood thinners, anti-coagulant medications, sometimes aspirin
Chemotherapy
Immunotherapy
Phlebotomy
Transformations from
None
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
Budd-Chiari syndrome
Deep vein thrombosis (DVT)
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
Myocardial ischemia or stroke
Reddened face that looks like a blush or sunburn
Splenomegaly
Stroke
Vascular abnormalities
Weakness
Weight loss for no reason
Recurrence and Metastases
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