SEER is an authoritative source of information on cancer incidence and survival in the United States. SEER currently collects and publishes cancer incidence and survival data from population-based cancer registries covering approximately 28 percent of the U.S. population.
9 - Grade/differentiation unknown, not stated, or not applicable
Chronic erythremia [OBS]
Polycythemia rubra vera
Polycythemia with chronic cyanosis
Spent phase polycythemia
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 hematopoieticstem cell, characterized by increased RBC production independent of mechanisms that normally regulate erythropoiesis.
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