Incurable Polycythemia Vera Causes Death from Blood Clots

Polycythermia vera is a rare and chronic blood disorder that occurs when the bone marrow produces too many red blood cells. It's the opposite of anemia, which results from too few red blood cells; it is also called plethora. When there are too many red blood cells, the blood thickens, causing circulatory-related problems. Polycythermia vera is incurable, affects more men than women, and rarely occurs before the age of 40.

Polycythermia vera is a myeloproliferative disorder, meaning the bone marrow is not only producing too many cells, but producing them too quickly. It develops slowly, over a period of many years, and there are no diagnosable symptoms specific to polycythermia vera. It is generally discovered through routine blood tests after a person seeks medical attention for other symptoms, such as fatigue, tingling sensations in hands and feet, headaches, ringing ears, breathing difficulties, lightheadedness, high blood pressure, unusually heavy bleeding from minor cuts, nosebleeds, stomach ulcers, etc. In short, it's often a diagnosis of exclusion.

As polycythermia vera progresses, some sufferers experience hemorrhage or blood clots. A small percentage of sufferers develop gout and ulcers. Blood clots are the most frequent cause of death from polycythermia vera.

In about a third of those with what is called late stage polycythermia vera, their bone marrow eventually fails. The person becomes anemic, the spleen and liver become enlarged, and blood transfusions become necessary.

Scientists are unsure of what causes polycythermia vera, but they have determined that it is not caused or triggered by other illnesses.

Once polycythermia vera is diagnosed, an individualized treatment plan is put in place to reduce the thickness of the blood and prevent hemorrhage and blood clots. Although thinning the blood sounds simple, care must be taken not to use drugs or over-the-counter medications such as aspirin that might also cause stomach ulcers. To begin with, most doctors will remove a pint of blood a week for a time to lower the red blood cell count (hematocrit). Withdrawn blood may be replaced with fluids to help dilute the red blood cell concentration. Once the hematocrit is less than 45, removal of blood will be done only as necessary. Other forms of polycythermia vera treatment may include partial transfusion and chemotherapy to suppress the bone marrow and to lower blood and platelet counts.

Polycythermia vera is a serious illness that can cause death. One of the biggest concerns for many sufferers is that it can sometimes evolve into acute leukemia. However, with new treatments and more on the way, people with polycythermia vera are living longer than ever before.