Blood Disorders Polycythemia Vera & Myelofibrosis An Overview of Secondary Polycythemia An Overproduction of Red Blood Cells Caused by Health Disorders By Deborah Leader, RN Updated on March 20, 2024 Medically reviewed by Steffini Stalos, DO Print KTS Design / Science Photo Library / Getty Images Table of Contents View All Table of Contents Symptoms Causes Diagnosis Treatment Coping Secondary polycythemia, similar to primary polycythemia vera, is a disorder that causes an overproduction of red blood cells. When too many red blood cells are produced, the blood becomes thick, hindering its passage through the smaller blood vessels. This article discusses the symptoms and causes of secondary polycythemia in addition to diagnosis and treatment. What is the difference between primary and secondary polycythemia? The difference between the two is that primary polycythemia starts inside the bone marrow and secondary polycythemia is associated with an underlying condition. Secondary Polycythemia Symptoms Symptoms of secondary polycythemia usually develop slowly over many years and are the same as those for primary polycythemia, such as: WeaknessHeadacheFatigueLightheadednessShortness of breathVisual disturbancesItching (pruritus)Pain in the chest or leg musclesRuddy complexionConfusionRinging in the ears (tinnitus)Burning sensations of the hands or feet What Is the Most Common Cause of Secondary Polycythemia? Primary polycythemia is caused by an abnormality of the cells in the bone marrow that form red blood cells. Secondary polycythemia is caused by a disorder originating outside of the bone marrow that causes overstimulation of the normal bone marrow, leading to an overproduction of red blood cells. The job of a red blood cell is to deliver oxygen to the body tissues. Smoking, for example, interferes with this process, so it is a common cause of secondary polycythemia. Others include: Brain, liver, or uterine tumors Chronic carbon monoxide exposure Chronic heart disease Chronic lung disease including chronic obstructive pulmonary disease (COPD) Diuretics Hypoventilation High altitude Kidney cysts Obesity Performance-enhancing drugs or blood doping in sports Sleep apnea How Is Secondary Polycythemia Diagnosed? Measuring oxygen levels in the blood with a blood test known as arterial blood gas (ABG) can help healthcare providers diagnose secondary polycythemia. Other blood tests include measurement of erythropoietin and red blood cell mass levels. Additional diagnostic tests include: Electrocardiogram (ECG) Chest X-ray Computed tomography (CT) scan Ultrasound These tests measure heart function and can detect the enlargement of the heart, liver, or spleen. Your healthcare provider may also use a genetic test that looks for a mutation in a gene called JAK2 that affects the bone marrow and blood cells. This test is positive in most cases of primary polycythemia and, as a result, is used to differentiate between primary polycythemia and secondary polycythemia. Treatment for Secondary Polycythemia Treatment for secondary polycythemia should control or eliminate the underlying condition. Symptom relief may include medications such as antihistamines to relieve itching, or aspirin to soothe pain and burning associated with the disorder. Because it takes time to treat the underlying condition, healthcare providers sometimes use phlebotomy (blood-letting) to reduce the number of red blood cells in plasma. As much as a pint (475 milliliters) may be taken in one sitting as if tolerated. Understanding Therapeutic Phlebotomy Coping With Secondary Polycythemia If you're already coping with one condition that can cause secondary polycythemia, such as COPD or a tumor, learning that you have a second diagnosis can feel discouraging or frustrating. Remember that secondary polycythemia is caused by an underlying condition, most of which are well known and have multiple treatment options available. Once the underlying cause is corrected, symptoms of secondary polycythemia usually go away. Your healthcare provider will guide you along the best path for resolving both. Summary Secondary polycythemia is associated with an underlying condition and causes an overproduction of red blood cells. Symptoms usually develop gradually and can include headache, weakness, pain, confusion, and more. Treating the underlying condition will also treat and correct polycythemia. Polycythemia Vera Healthcare Provider Discussion Guide Get our printable guide for your next healthcare provider's appointment to help you ask the right questions. Download PDF Sign up for our Health Tip of the Day newsletter, and receive daily tips that will help you live your healthiest life. Email Address Sign Up You're in! Thank you, {{form.email}}, for signing up. There was an error. Please try again. 4 Sources Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy. McMullin MFF, Mead AJ, Sahra A, et al. A guideline for the management of specific situations in polycythaemia vera and secondary erythrocytosis: a British Society for Haematology guideline. Br J Haematol. 2019 Jan;184(2):161-75. doi:10.1111/bjh.15647 National Organization of Rare Diseases. Polycythemia vera. Haider MZ, Anwer F. Secondary Polycythemia. [Updated 2023 May 8]. Assi TB, Baz E. Current applications of therapeutic phlebotomy. Blood Transfus. 2014 Jan;12(Suppl 1):s75-83. doi:10.2450/2013.0299-12 By Deborah Leader, RN Deborah Leader RN, PHN, is a registered nurse and medical writer who focuses on COPD. 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