Polycythemia can present with the following signs and symptoms:

• Feeling of malaise or general weakness
• Headache
• Vertigo and dizziness [7]
• Tinnitus
• Dyspnea
• Pruritus
• Weight loss
• Constipation
• Splenomegaly
• Arthritis
• Redness in skin in areas of hands, feet and face [8]
• High blood pressure
• Epistaxis

• Anemia

  Erythroblastosis, which indicates chronic anemia, is also a good diagnostic indicator of TAPS. [ncbi.nlm.nih.gov]

  ( D50 - D64 ) anemia of premature infant ( P61.2 ) benign (familial) polycythemia ( D75.0 ) congenital anemias ( P61.2- P61.4 ) newborn anemia due to isoimmunization ( P55.- ) polycythemia neonatorum ( P61.1 ) polycythemia NOS ( D75.1 ) polycythemia [icd10data.com]

• Splenomegaly

  Splenomegaly Massive splenomegaly is the most difficult complication of polycythemia vera to manage, since it is usually refractory to chemotherapy and not durably alleviated by irradiation. [cancertherapyadvisor.com]

  Patient demographics, laboratory data, and the presence of splenomegaly by disease feature including known HU use, known phlebotomy requirements, and the presence of splenomegaly were thoroughly assessed. [medpagetoday.com]

  The hematological features include the presence of isolated erythrocytosis without evolution into leukemia or other myeloproliferative disorders, absence of splenomegaly, normal white blood cell and platelet counts, and low plasma EPO levels. [orpha.net]

  Splenomegaly and pruritus may persist despite control of the haematocrit by phlebotomy[10]. [patient.info]

  Patients were required to have splenomegaly and need phlebotomy for adequate Hct control. [link.springer.com]

• Massive Splenomegaly

  Although the blood count normalised there was no effect on the massive splenomegaly. So, a splenectomy was performed. [openaccesspub.org]

  Splenic embolization for massive splenomegaly is not recommended because of the toxicity associated with the attendant spleen necrosis. [cancertherapyadvisor.com]

  defined as either an increase in palpable splenomegaly of > 5.0 cm from baseline (distance from left costal margin to the tip of the spleen) or appearance of newly palpable splenomegaly At least 2 of these constitutional symptoms: >10% weight loss in [pathologyoutlines.com]

• Diarrhea

  Causes of spurious polycythemia include: burns diarrhea hemoconcentration (higher-than-normal concentration of cells and solids in the blood, usually due to becoming dehydrated or taking diuretics ) stress Weakness, headaches, and fatigue are usually [encyclopedia.com]

  Relative polycythemia may be the consequence of abnormally lowered fluid intake or of marked loss of body fluid, such as occurs in persistent vomiting, severe diarrhea, or copious sweating or when water is caused to shift from the circulation into the [britannica.com]

  […] nausea, insomnia, body pain Confusion, depression, autoimmunity, hyperlipidemia Psychiatric disease, cardiovascular disease Radioactive phosphorus ( 32 P) Radiopharmaceutical Anemia, thrombocytopenia, leukopenia, Leukemia may develop after treatment Diarrhea [aafp.org]

• Vomiting

  There is an approximately 50% chance of nausea and vomiting which would be self-limited. Female patients who may be pregnant should not undergo this therapy. [radiology.uic.edu]

  Hepatic venous thrombosis will present with abdominal distension(ascites), features of liver insufficiency like nausea, vomiting, loss of appetite, yellowish discoloration of eyes, generalized swelling and gastrointestinal bleeding( either as haemoptysis [medicaljoyworks.com]

  Side effects include fever, weakness, nausea, and vomiting which limits the useful of this medication. Busulfan for PV For patients that have failed hydroxyurea and/or interferon alpha, busulfan, a chemotherapeutic medication, may be used. [verywellhealth.com]

  Relative polycythemia may be the consequence of abnormally lowered fluid intake or of marked loss of body fluid, such as occurs in persistent vomiting, severe diarrhea, or copious sweating or when water is caused to shift from the circulation into the [britannica.com]

• Abdominal Fullness

  If a person has developed polycythemia as a result of liver cancer, kidney cancer, or other erythropoietin-secreting tumors, then symptoms include weight loss, abdominal fullness or pain and jaundice. [epainassist.com]

  If treatment is possible, the person should make a full recovery. In cases of PV, treatment will focus on managing the condition and symptoms, as there is currently no cure. [medicalnewstoday.com]

• Hepatomegaly

  Physical findings include plethora, splenomegaly, and, in 25% of cases, hepatomegaly as well as those associated with the complications. [annals.org]

   Plethoric face  Splenomegaly –75%  Hepatomegaly –30% Physical 15.  Polymerase chain reaction :JAK2 mutation  Serum Epo assay: are often below the lower limit of normal.  Leukocyte alkaline phosphatase distinguises PV from CML. [slideshare.net]

  Nonspecific neurological symptoms : dizziness, headache, visual disturbances, tinnitus Hypertension Splenomegaly : associated with early satiety and abdominal discomfort; less commonly hepatomegaly Peptic ulcer disease Symptoms of thrombotic and hemorrhagic [amboss.com]

  Hepatomegaly absent absent present in 35% of the cases 10. Heart or lung disease absent present absent 11. Cyanosis may be present present absent Possible References Used Back to tests [labpedia.net]

  Less common symptoms associated with polycythemia vera include a tendency to bruise easily, frequent nosebleeds or bleeding from the gums, enlargement of the liver (hepatomegaly), and erythromelalgia, a condition characterized by a reddened or purplish [rarediseases.org]

• Thrombosis

  thrombosis (portal vein thrombosis and Budd-Chiari syndrome; see these terms) and bleeding including gum bleeding, ecchymoses and gastrointestinal bleeding. [orpha.net]

  Unusual sites of venous thrombosis are more common in patients with myeloproliferative neoplasms, including splanchnic vein thrombosis (SVT) and cerebral vein thrombosis. [ajmc.com]

  Hyperviscosity is a clinical syndrome involving sludging of blood within vessels and sometimes thrombosis. [msdmanuals.com]

• Hypertension

  KEYWORDS: erythropoietin; polycythemia; pulmonary hypertension; von Hippel-Lindau gene [ncbi.nlm.nih.gov]

  Two affected individuals developed severe pulmonary hypertension, a hitherto unrecognized consequence of mutations in the pathway. [doi.org]

  : Plasma volume in men with essential hypertension. N. Engl. J. Med., 278: 762, 1968. [ang.sagepub.com]

• Heart Disease

  […] bronchitis, emphysema Chronic heart disease like congestive heart failure Cyanotic heart disease (congenital) Sleep apnea Obesity (Pickwickian syndrome) Abnormally High Erythropoietin There may be various causes of inappropriately high erythropoietin [healthhype.com]

  Venesection is not performed in cyanotic heart disease except at the request of the cardiologist. Where testosterone replacement must be continued it is appropriate to refer for venesection. [rms.kernowccg.nhs.uk]

  Secondary polycythemia, which is caused by factors that reduce the amount of oxygen reaching the body's tissues, such as smoking, high altitude, or congenital heart disease. [dana-farber.org]

• Osteoporosis

  Comprehensive health information about cancer, osteoporosis, high blood pressure and more. Comprehensive health information including health news updates and health resources. [web.archive.org]

• Flushing

  Clinical features include facial flushing, pruritus, erythromelalgia, headaches, and dizziness. Severe complications such as thrombotic events (e.g., stroke, Budd-Chiari syndrome) may occur. [amboss.com]

  As the disease progresses, symptoms include: Shortness of breath Dizziness Itchy or flushed skin Fatigue Headaches A tingling or burning sensation in the hands and feet What causes polycythemia vera? PV begins in the bone marrow. [familydoctor.org]

  If the skin is flushed or itchy, particularly after a hot bath, it may indicate a diagnosis of polycythemia. The doctor may also check for a history of smoking or alcohol abuse or a family history of kidney disease. [chealth.canoe.com]

  Symptoms Symptoms of PV may include: Fatigue and/or weakness Headache Dizziness Shortness of breath and difficulty breathing while lying down Visual disturbance, such as double vision, blurred vision, and blind spots Inability to concentrate Night sweats Flushed [hopkinsmedicine.org]

• Purpura

  Dr Nina Salooja Haematologist (blood specialist) (1) Bleeding disorders, Thrombosis, Anaemia, Purpura, Haemochromatosis, Platelets disorders Loading... [topdoctors.co.uk]

  Additionally, symptoms of petechia, purpura, ecchymosis, splenomegaly, hepatomegaly, peptic ulcers and hyperuricaemia are seen [ 6, 7 ]. [omicsonline.org]

• Headache

  The presenting symptoms are frequently vague and consist of fatigue, headaches, dizziness, bleeding, pruritus, dyspepsia, and thrombosis. [medlink.com]

  Clinical features include facial flushing, pruritus, erythromelalgia, headaches, and dizziness. Severe complications such as thrombotic events (e.g., stroke, Budd-Chiari syndrome) may occur. [amboss.com]

  Share on Pinterest A person with polycythemia may experience headaches, dizziness, and excessive sweating. There are two types of polycythemia, which have different causes. [medicalnewstoday.com]

  On review of systems, he reveals that he is fatigued and experiences night sweats and regular headaches. His weight today is 20 lbs lighter than his previous documented weight. [step2.medbullets.com]

  Headaches and dizziness due to frequent bouts of high blood pressure may also be observed. Blood is reguarly removed from the patients of polycthemia to keep the red cell counts at an acceptable level. [symptoma.com]

• Stroke

  Default of therapy for polycythemia is associated with a profound risk of recurrent strokes requiring patient education and support to prevent the devastating consequence of this modifiable but rare cause of stroke. [ncbi.nlm.nih.gov]

  It can cause blood clots resulting in a heart attack, stroke, or pulmonary embolism. Liver and spleen enlargement are other possible complications. [hopkinsmedicine.org]

  There is a marked increase in stroke and other thrombotic events in these patients, although thrombosis does not account for all of the increased mortality. [hematologyandoncology.net]

• Dizziness

  The presenting symptoms are frequently vague and consist of fatigue, headaches, dizziness, bleeding, pruritus, dyspepsia, and thrombosis. [medlink.com]

  Clinical features include facial flushing, pruritus, erythromelalgia, headaches, and dizziness. Severe complications such as thrombotic events (e.g., stroke, Budd-Chiari syndrome) may occur. [amboss.com]

  Lethargy, confusion, headache, dizziness These symptoms are due to central nervous system disturbances with hyperviscosity causing poor perfusion Lethargy, confusion, headache, dizziness These symptoms are due to central nervous system disturbances with [medicaljoyworks.com]

  Headaches and dizziness due to frequent bouts of high blood pressure may also be observed. Blood is reguarly removed from the patients of polycthemia to keep the red cell counts at an acceptable level. [symptoma.com]

  Do you sometimes feel dizzy or weak? Have you had shortness of breath? Do you sweat a lot at night? You may get some blood tests, too. These include: Complete blood count (CBC). [webmd.com]

• Vertigo

  Clinical description Symptoms are often insidious at onset and may include headache, dizziness, vertigo, tinnitus, visual disturbances, and pruritus after bathing, a ruddy complexion that manifests in the face, palms, nailbeds, mucosa and conjunctiva. [orpha.net]

  A 24-year-old man presented in March, 2000, complaining of asthenia, without headache, aquagenic pruritus, tinnitus, or vertigo. [thelancet.com]

   Symptoms are related to 1. hyperviscosity 2. sludging of blood flow 3. thromboses  which lead to poor oxygen delivery and symptoms that include headache, dizziness, vertigo, tinnitus, visual disturbances, angina pectoris, or intermittent claudications [slideshare.net]

  Many patients have neurologic symptoms such as headaches, dizziness or vertigo, visual problems, and paresthesias at the time of diagnosis. [medlink.com]

Polycythemia is diagnosed on the basis of clinical features and laboratory findings. The following tests are used in the work up of patients with polycythemia:

• Complete blood count (CBC): This determines the red cell mass, red cell count and hematocrit among suspected patients. Thrombocyte count and leukocyte count can also be derived from this laboratory test.
• Leukocyte alkaline phosphatase level: The leukocyte alkaline phosphatase level is generally elevated up to 100U/L in almost 70% of patients of polycythemia. 
• Routine coagulation tests: Prothrombin time (PT) and activated partial thromboplastin time (aPTT) are temporarily lengthened in polycythemic patients. 
• Imaging studies: The use of ultrasonography and computed tomography (CT scan) reveals the presence of an splenomegaly in the patients of polycythemia.

• Decreased Plasma Volume

  There is pseudopolycythemia where red cell volume is normal but the plasma volume is reduced. [labpedia.net]

  Apparent polycythaemia is caused by a decreased plasma volume. All of these are reasons not to accept a donation, either because of the association with malignancy, or because of the potential to harm the donor. [transfusionguidelines.org]

  Isotope dilution techniques to determine the red cell mass and plasma volume can differentiate true erythrocytosis from a spurious elevation due to a decrease in plasma volume. 6,7 However, this is an expensive, time-consuming test that is not widely [mdedge.com]

  Relative polycythemia, or erythrocythemia, results from decreased plasma volume. A true polycythemia or erythrocythemia results from increased red blood cell mass. [emedicine.medscape.com]

• Thrombocytosis

  For example, isolated thrombocytosis, associated with a JAK2 mutation, can be the presenting manifestation of essential thrombocytosis, primary myelofibrosis, or polycythemia vera, while both essential thrombocytosis and primary myelofibrosis can evolve [cancertherapyadvisor.com]

  For patients with marked thrombocytosis the administration of anagrelide may be indicated. Although it is not a curable disease, it is, because of its chronic nature, usually effectively managed over long periods of time. [fcarreras.org]

  Polycythemia is frequently used synonymously with erythrocytosis; however, polycythemia may imply leukocytosis and thrombocytosis, as well as erythrocytosis. Erythrocytosis and Polycythemia Overview of Erythrocytosis and Polycythemia [msdvetmanual.com]

  Definition / general Also referred to as polycythemia rubra vera, primary polycythemia and essential polycythemia BCR-ABL1 negative myeloproliferative neoplasm (MPN) characterized by peripheral erythrocytosis with or without leukocytosis or thrombocytosis [pathologyoutlines.com]

  Alternative form: polycythaemia Synonyms: erythrocytosis erythrocythemia hypercythemia hypererythrocythemia Compare: anemia, leukocytosis, thrombocytosis. [biology-online.org]

• Hyperviscosity

  Diagnosis of hyperviscosity syndrome is clinical. [msdmanuals.com]

  The elevated blood cell mass results in hyperviscosity, which is associated with slow blood flow and an increased risk of thrombosis. The disorder typically initially has a long asymptomatic period, then assumes a chronic, insidious course. [amboss.com]

   Symptoms of PV are often insidious in onset, and they are often related to blood hyperviscosity.  Symptoms are related to 1. hyperviscosity 2. sludging of blood flow 3. thromboses  which lead to poor oxygen delivery and symptoms that include headache [slideshare.net]

  In the neonatal period, polycythemia-induced hyperviscosity can lead to altered blood flow and can subsequently affect organ function. [emedicine.medscape.com]

• Cytopenia

  “The criteria are becoming more sensitive,” which is important, but even this doesn’t tell the whole story, Mesa noted, because PV carries a heavy symptom burden, including vascular events, cytopenias, and splenomegaly, along with a risk of progression [curetoday.com]

  Most adverse events were grade 1 or 2, and there were few grade 3 or 4 cytopenias. As reported in a prior analysis, the rate of herpes zoster was higher in the ruxolitinib arm. The RESPONSE study was conducted at 90 sites worldwide. [medicalnewstoday.com]

  Palpable splenomegaly is one of the worst offenders since it contributes to early satiety, bloating, pain, portal hypertension, weight loss, and exacerbation of cytopenia. [medpagetoday.com]

  These diagnostic criteria are directed primarily at the exclusion of other myeloproliferative neoplasms (MPN) and of other clinical conditions that could account for the cytopenias. [openaccesspub.org]

Polycythemia can be treated symptomatically by reducing the red blood cell mass by phlebotomy or the physical drainage of blood from a given vein. Patients who have undergone phlebotomy have a decreased chance of developing a thrombosis in the near future [9]. In patients with polycythemia vera, regular phlebotomy may be indicated because of the chronic nature of the disease.

Patients with thicker blood viscosity benefit from the regular intake of low dose aspirin to prevent blood clots. The use of aspirin also addresses the burning sensation of the hands and feet felt by the patients suffering from polycythemia.

Patients are sometimes given hydroxyurea and interferon to control the overproduction of blood cells in the bone marrow [10].

Anti-itch medications like oral anti-histamines may be given to control the itching felt in the palms and soles of patients. Ultraviolet light (UV) has been arbitrarily used to relieve the itch symptoms in the skin.

Polycythemia is a type of chronic disease requiring long term treatment regimen. Individuals who strictly adhere to the treatment can live a healthy life without any complications. Patients with secondary polycythemia (like smokers and asthmatics) have a greatly reduced the risk for cardiovascular events when regular phlebotomy is done.

Polycythemia can either be congenital in nature or can be acquired. The primary type of polycythemia occurs when there is a overproduction of red blood cells by the bone marrow. The most common type of primary polycythemia is known as polycythemia vera. Secondary polycythemia occurs due to certain underlying diseases.

Various factors predispose individuals to developing relative polycythemia. These include obesity, smoking, dehydration and medications such as diuretics and excessive consumption of alcohol [2].

Primary polycythemia is a rare occurrence. It has been estimated that in the United States, there are just about 45 - 57 cases per 100,000 population [3]. Individuals above 60 years of age are at an increased risk of contracting this blood condition. The incidence of primary polycythemia, in individuals younger than 20 years is estimated to be about 0.01%.
Studies have shown that males are more prone to contract this condition than females, with the male to female ratio being 1.2:2.2 [4].

Mutations that are either acquired or inherited are the primary factors that favor the development of primary polycythemia. Polycythemia vera, which is the most common form of primary polycythemia, occurs as a result of excessive production of red blood cells (RBC's) due to certain abnormalities in the bone marrow [5]. It is a type of myoproliferative disease which is characterized by increased production of myeloid cells [6]. In such cases, excess production of RBC's is also accompanied by excessive production of white blood cells (WBC's) and platelets (thrombocytes). 

Secondary form of polycythemia usually occurs due to an underlying disease condition and is characterized by 6 - 8 million erythrocytes per cubic millimeter of blood. Another factor which greatly triggers the development of polycythemia is the state of reduced oxygen supply, typically seen among chronic smokers and asthmatics. Such conditions cause an increased production of RBC's causing the development of polycythemia.

Patients diagnosed with polycythemia should have regular exercises to prevent blood clot formation and improve blood vessel circulation in the body. Tobacco should be strictly prohibited to prevent polycythemia from recurring. Polycythemic patients are prone to the development of sores in hands and feet. A good wound care is imperative to prevent complications especially in tissues with poor blood circulation.

The condition develops either due to increase in red blood cell count or decrease in plasma volume. In the former case, the condition is referred to as absolute polycythemia; whereas in the latter, it is referred to as relative polycythemia.

Absolute polycythemia is divided into 2 categories; namely, primary and secondary polycythemia. Both of these forms can either be congenital or acquired [1].

Polycythemia is a disorder characterized by an abnormally increased number of red blood cells in the blood. Polycythemia may either occur at birth or later in life secondary to conditions such as smoking, dehydration and obesity. Patients of polycythemia perennially complain of fatigue and disturbed balance. Itching is also a common feature. Headaches and dizziness due to frequent bouts of high blood pressure may also be observed. Blood is reguarly removed from the patients of polycthemia to keep the red cell counts at an acceptable level. Medicines are given to prevent the formation of blood clots and to relieve the symptoms of itching.

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