Polycythemia vera (pol-e-sy-THEE-me-uh VEER-uh) is a type of blood cancer. It causes your bone marrow to make too many red blood cells. These excess cells thicken your blood, slowing its flow, which may cause serious problems, such as blood clots. Show
Polycythemia vera is rare. It usually develops slowly, and you might have it for years without knowing. Often the condition is found during a blood test done for another reason. Without treatment, polycythemia vera can be life-threatening. But proper medical care can help ease signs, symptoms and complications of this disease. Products & Services
SymptomsMany people with polycythemia vera don't have noticeable signs or symptoms. Some people might develop vague symptoms such as headache, dizziness, fatigue and blurred vision. More-specific symptoms of polycythemia vera include:
When to see a doctorMake an appointment with your doctor if you have signs or symptoms of polycythemia vera. Request an Appointment at Mayo Clinic There is a problem with information submitted for this request. Review/update the information highlighted below and resubmit the form. Sign up for free, and stay up to date on research advancements, health tips and current health topics, like COVID-19, plus expertise on managing health. Email ErrorEmail field is required ErrorInclude a valid email address Learn more about Mayo Clinic’s use of data.To provide you with the most relevant and helpful information, and understand which information is beneficial, we may combine your email and website usage information with other information we have about you. If you are a Mayo Clinic patient, this could include protected health information. If we combine this information with your protected health information, we will treat all of that information as protected health information and will only use or disclose that information as set forth in our notice of privacy practices. You may opt-out of email communications at any time by clicking on the unsubscribe link in the e-mail. Clinical features vary according to the origin of the disease but may include plethora or ruddy complexion, headache and tinnitus. The congenital form may be complicated by superficial or deep vein thrombophlebitis, or present with associated symptoms as in Chuvash erythrocytosis (see this term), or the course of the disease may be indolent. Patients with a specific sub-type of congenital secondary polycythemia, known as Chuvash erythrocytosis, present with lower systolic or diastolic blood pressure, venous varicosities, and vertebral body hemangiomas and may be complicated by cerebrovascular events or mesenteric thrombosis. The acquired form of secondary polycythemia may present with cyanosis, hypertension, clubbing of the fingers and toes and lethargy. EtiologySecondary polycythemia may be congenital and caused by defects in the oxygen sensing pathway due to autosomal recessive mutations in the VHL, EGLN1 and EPAS1 genes (3p26-p25, 1q42-q43 and 2p21-p16 respectively) which result in enhanced erythropoietin (EPO) production in hypoxic conditions, or by other autosomal dominant congenital defects including high oxygen-affinity hemoglobin and biphosphoglycerate mutase deficiency, which result in tissue hypoxia and a secondary erythrocytosis. Alternatively, secondary polycythemia may be acquired and caused by increased amounts of EPO. This may be due to tissue hypoxia that may be central and caused by pulmonary or cardiac disease or high altitude, or local and caused by hypoxia in the kidney such as renal artery stenosis. EPO production may be pathologic and caused by EPO secreting tumors such as renal cell cancer, hepatocellular carcinoma, cerebellar hemangioblastoma, meningioma and parathyroid carcinoma/adenoma (see these terms). In addition EPO may be administered deliberately to produce erythrocytosis and enhance performance. Diagnostic methodsDiagnosis is based on evidence of increased total red blood cells and normal to high serum EPO levels. Secondary causes of erythrocytosis must be diagnosed individually and will require a comprehensive history. Differential diagnosisDifferential diagnoses include polycythemia vera and primary familial polycythemia (see these terms), which can be excluded on the basis of low EPO levels and the presence of a mutation in the JAK2 gene (9p24) for polycythemia vera. Management and treatmentThere is little evidence to guide management of congenital secondary polycythemia. Phlebotomy or venesection may be of benefit, particularly in patients at increased risk of thrombosis. A target hematocrit (Hct) of 50% may be the most practical. In patients with no specific contraindication, low-dose aspirin may be of benefit. In acquired cases of secondary polycythemia, management is based on treating the underlying condition. PrognosisPrognosis depends mainly on the associated disease in the acquired forms of secondary erythrocytosis and on the severity of thrombotic complications in the inherited forms such as in Chuvash erythrocytosis. The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. What is secondary polycythemia symptoms?Symptoms of Secondary Polycythemia
Patients with secondary polycythemia may have symptoms including: Fatigue. Headache. Dizziness. Blurred vision.
What is the most common cause of polycythemia?What causes polycythemia vera? Polycythemia vera is caused by a genetic change (mutation) that develops during your lifetime. It is not an inherited genetic disorder. In most cases it is not known why this happens.
Which type of complication is most common in patients with polycythemia?Polycythemia Vera Complications
Blood clots are the most serious complication of PV. Blood clots can cause a heart attack or stroke. They also can cause your liver and spleen to enlarge. Blood clots in the liver and spleen can cause sudden, intense pain.
What is the disease of polycythemia?Overview. Polycythemia vera (pol-e-sy-THEE-me-uh VEER-uh) is a type of blood cancer. It causes your bone marrow to make too many red blood cells. These excess cells thicken your blood, slowing its flow, which may cause serious problems, such as blood clots.
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