Polycythemia vera is one of the primary types of chronic myeloproliferative disorders. In this disease, an excessive number of red blood cells develop in the blood and bone marrow. An overproduction of platelets and white blood cells may also be present. Symptoms include thickened blood, abnormal bleeding, and an enlarged spleen. Treatment options include chemotherapy, phlebotomy, low-dose aspirin, and others.
There are no screening tests for myelodysplastic syndromes. However, doctors can detect blood cell abnormalities when performing routine blood work. In this case, you will get further testing.
1 AnswerRealAge answeredPolycythemia vera (PV) is a rare blood disease in which your body makes too many red blood cells, and in some cases too many white blood cells and platelets as well. This overproduction of cells can cause the blood to become unusually thick, making it move more slowly than normal throughout the body. Red blood cells carry oxygen throughout the body and if the blood flow is too slow, vital organs, including the heart, may not get enough oxygen and may become damaged. Thick blood is also more prone to producing blood clots and raising risk of heart attack or stroke.
PV is caused by a mutation on a specific gene that makes a protein that helps the bone marrow produce blood cells. The cause of the genetic mutation is unknown, but PV is not thought to be inherited or to run in families. PV is most likely to strike people age 60 or older and occurs more often in men than in women.
In its early stages, PV is usually symptomless. The disease is often detected on a blood test done for other reasons, before symptoms are even apparent. As the disease progresses, symptoms may include:
- shortness of breath
- skin itching
- blurred or double vision
- itchy or red skin, or red skin spots
- excessive bleeding
Past chemotherapy and exposure to certain chemicals including benzene can increase your risk of myelodysplastic syndromes. Some of the chemotherapy drugs that lead to the development of myelodysplastic syndromes are necessary in the treatment of certain cancers. The doctor may decide to use these drugs if the benefits seem to outweigh costs. You can decrease your chances of getting myelodysplastic syndromes by not smoking and by avoiding benzene or other hazardous chemicals.
1 AnswerDr. Stuart A. Linder, MD , Plastic Surgery, answeredPatients with Von Willebrand disease may have surgery after evaulated by a Hematologist and given the appropriate pre surgical medications. The Von Willebrand factor is the essential adherence substance or factor that binds platelets together for clotting. A factor 8 deficency is seen in these patients. Symptoms include bleeding from the gums, easily bruised, heavy periods, frequent nasal bleeds, bleeding after aspirin or related drugs. The hematology specialist will normally place the patient on DDAVP intravenously prior to surgery to increase your own production of von Willebrand factor.
1 AnswerRealAge answeredVon Willebrand disease is the most common inherited bleeding disorder. It occurs in people who either have a deficiency of a blood clotting protein called von Willebrand factor, or they have the clotting factor, but it malfunctions. Von Willebrand factor helps blood platelets clump together and stick to blood vessel walls, essential steps in normal blood clotting. Von Willebrand disease affects about 1 percent of Americans and occurs with equal frequency in men and women.
Symptoms of von Willebrand disease include:
- heavy, long menstrual periods (menorrhagia -- the most common symptom in women)
- bleeding gums
- excessive or prolonged bleeding after surgery or dental work
- excessive or prolonged bleeding (hemorrhaging) after giving birth
- bruising easily
- skin rash
Type 1 affects 60-80 percent of people with von Willebrand disease, is caused by having too little von Willebrand factor, and causes symptoms that are usually mild.
Type 2 affects 15-30 percent of people with von Willebrand disease, is due to malfunctioning von Willebrand factor, and causes mild to moderate symptoms.
Type 3 affects 5 to 10 percent of people with von Willebrand disease, is caused by a deficiency of von Willebrand factor, and can produce severe symptoms.
Acquired von Willebrand disease, the only type that is not inherited, can occur after the diagnosis of certain diseases (lupus, heart disease, some cancers) or as a result of taking certain medications.
Von Willebrand is treatable with certain prescription medications.
Bleeding, infections, and anemia are all potential complications of myelodysplastic syndromes. When the cells in your bone marrow malfunction, you become unable to produce adequate numbers of healthy blood cells. You may be prone to infections if you do not have enough healthy white blood cells. Inadequate platelet counts can lead to bleeding or bruising. You should talk to your doctor if you are concerned about complications, as there are treatments and preventative measures available.
The term "pre-leukemia" has been used to describe myelodysplastic syndromes. This is misleading, as most cases do not become cancerous. If you have mild disease according to the International Prognostic Scoring System, your risk of leukemia may only be 15 percent, whereas if you have severe disease, you may have nearly a 100 percent risk. Your doctor can help you figure out your prognosis and your likelihood of getting leukemia.
Factors affecting the prognosis of myelodysplastic syndromes include the number of normal blood cells in your peripheral circulation, the relative number of blasts in your bone marrow, and the presence of any anomalies in your chromosomes. The International Prognostic Scoring System, abbreviated IPSS, uses those factors in the determination of prognosis. Blasts are blood cells that have not matured properly and if you have a higher percentage of blasts, your prognosis is poorer. Those considered low risk by the International Prognostic Scoring System have a five year survival of 55 percent and a 15 percent risk of getting leukemia. Those considered high risk have a five year survival of zero, and a 100 percent risk of getting leukemia.
Myelodysplastic syndromes affect the ability of the bone marrow to effectively produce normal blood cells. If you do not have enough red blood cells, you may feel tired and you may notice that your skin looks pale. Platelets help your blood to clot so that you do not bleed to death from small cuts. If you do not have enough platelets, you may notice that small cuts bleed more than usual, or that you bruise after only mild trauma. Too few white blood cells can lead to infection, as your body is less able to combat viruses and bacteria.