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Encyclopedia > Polycythemia vera
Polycythemia vera
Classification & external resources
ICD-10 D45.
ICD-9 238.4
ICD-O: 9950/3
MeSH D011087

Primary polycythemia, often called polycythemia vera (PCV), polycythemia rubra vera (PRV), or erythremia, occurs when excess red blood cells are produced as a result of an abnormality of the bone marrow. Often, excess white blood cells and platelets are also produced. Polycythemia vera is classified as a myeloproliferative disease. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) is a coding of diseases and signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or diseases, as classified by the World Health Organization (WHO). ... // C00-D48 - Neoplasms (C00-C14) Malignant neoplasms, lip, oral cavity and pharynx (C00) Malignant neoplasm of lip (C01) Malignant neoplasm of base of tongue (C02) Malignant neoplasm of other and unspecified parts of tongue (C03) Malignant neoplasm of gum (C04) Malignant neoplasm of floor of mouth (C05) Malignant neoplasm of... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The International Classification of Diseases for Oncology (ICD-O) is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ... Polycythemia is a condition in which there is a net increase in the total number of red blood cells in the body. ... This article does not cite any references or sources. ... A scanning electron microscope image of normal circulating human blood. ... A 250 ml bag of newly collected platelets. ... The myeloproliferative diseases (MPDs) are a group of diseases of the bone marrow in which excess cells are produced. ...

Contents

Epidemiology

Polycythemia vera occurs in all age groups (including children),[1] although the incidence increases with age. One study found the median age at diagnosis to be 60 years,[2] while a study in Olmsted County, Minnesota found that the highest incidence was in people aged 70–79 years.[3] The overall incidence in the Minnesota population was 1.9 per 100,000 person-years, and the disease was more common in men than women.[3] Olmsted County is a county located in the U.S. state of Minnesota. ...


Symptoms

Patients with polycythemia vera are often asymptomatic. A classic symptom of polycythemia vera is generalized itching, particularly after exposure to warm water, which may be due to abnormal histamine release[4][5] or prostaglandin production.[6] Such itching is present in approximately 40% of patients with polycythemia vera.[2] Gouty arthritis may be present in up to 20% of patients.[2] Peptic ulcer disease is also common in patients with polycythemia vera; the reasons for this are unclear, but may be related to an increased susceptibility to infection with the ulcer-causing bacterium H. pylori.[7] In medicine, a disease is asymptomatic when it is at a stage where the patient does not experience symptoms. ... Pruritis is a misspelling of the medical condition pruritus. ... This article or section does not cite any references or sources. ... Chemical structure of prostaglandin E1 (PGE1). ... Gout (also called metabolic arthritis) is a disease due to a congenital disorder of uric acid metabolism. ... Peptic ulcer is a non-malignant ulcer of the stomach (called gastric ulcer) or duodenum (called duodenal ulcer). ... Binomial name Helicobacter pylori ((Marshall 1985) Goodwin 1989) Helicobacter pylori is a helical shaped Gram-negative bacterium that colonises the mucus layer of gastric epithelium in the stomach, and also the duodenum when it has undergone gastric metaplasia. ...


A rare but classic symptom of polycythemia vera (and the related myeloproliferative disease essential thrombocythemia) is erythromelalgia.[8] This is a sudden, severe burning pain in the hands or feet, usually accompanied by a reddish or bluish coloration of the skin. Erythromelalgia is caused by an increased platelet count or increased platelet "stickiness", resulting in the formation of tiny blood clots in the vessels of the extremity; it responds rapidly to treatment with aspirin.[9][10] Essential thrombocytosis (ET, essential thrombocythemia) is a rare and chronic blood disorder characterized by the overproduction of megakaryocytes (the precursor cell for platelets. ... Erythromelalgia, also known as Mitchells disease (after Silas Weir Mitchell) and red neuralgia(also referred to as man on fire), is a rare disorder in which blood vessels, usually in the extremities and especially in the feet, are blocked and inflamed, causing a painful burning and throbbing sensation and... Cyanosis refers to the bluish coloration of the skin due to the presence of deoxygenated hemoglobin in blood vessels near the skin surface. ... Aspirin, or acetylsalicylic acid (IPA: ), (acetosal) is a drug in the family of salicylates, often used as an analgesic (to relieve minor aches and pains), antipyretic (to reduce fever), and as an anti-inflammatory. ...


Patients with polycythemia vera are prone to the development of blood clots (thrombosis). A major thrombotic complication (e.g. heart attack, stroke, deep venous thrombosis, or Budd-Chiari syndrome) may sometimes be the first symptom or indication that a person has polycythemia vera. Thrombosis is the formation of a clot or thrombus inside a blood vessel, obstructing the flow of blood through the circulatory system. ... Acute myocardial infarction (AMI or MI), more commonly known as a heart attack, is a disease state that occurs when the blood supply to a part of the heart is interrupted. ... Stroke (or cerebrovascular accident or CVA) is the clinical designation for a rapidly developing loss of brain function due to an interruption in the blood supply to all or part of the brain. ... It has been suggested that Deep Vein Thrombosis be merged into this article or section. ... In medicine (gastroenterology and hepatology), Budd-Chiari syndrome is the clinical picture caused by occlusion of the hepatic vein. ...


Diagnosis

Patients with polycythemia vera may often be asymptomatic. Physical exam findings are non-specific, but may include enlarged liver or spleen, plethora, or gouty nodules. The diagnosis is often suspected on the basis of laboratory tests. Common findings include an elevated hemoglobin level or hematocrit, reflecting the increased number of red blood cells; the platelet count or white blood cell count may also be increased. Because polycythemia vera results from an essential increase in erythrocyte production, patients have a low erythropoietin (EPO) level. In medicine, a disease is asymptomatic when it is at a stage where the patient does not experience symptoms. ... Hepatosplenomegaly is the simultaneous enlargement of both the liver (hepatomegaly) and the spleen (splenomegaly). ... A tophus (Latin: stone, plural tophi) is a deposit of crystallised monosodium urate in people with longstanding hyperuricemia. ... The hematocrit (Ht or HCT) and packed cell volume (PCV) are measures of the proportion of blood volume that is occupied by red blood cells. ... A 250 ml bag of newly collected platelets. ... Schematics of shorthand for complete blood count commonly used by physicians. ...


In primary polycythemia, there may be 8 to 9 million and occasionally 11 million erythrocytes per cubic millimeter of blood (a normal range for adults is 4-6), and the hematocrit may be as high as 70 to 80%. In addition, the total blood volume sometimes increases to as much as twice normal. The entire vascular system can become markedly engorged with blood, and circulation times for blood throughout the body can increase up to twice the normal value. The increased numbers of erythrocytes can cause the viscosity of the blood to increase as much as five times normal. Capillaries can become plugged by the very viscous blood, and the flow of blood through the vessels tends to be extremely sluggish. The hematocrit (Ht or HCT) and packed cell volume (PCV) are measures of the proportion of blood volume that is occupied by red blood cells. ... Human red blood cells Red blood cells are the most common type of blood cell and are the vertebrate bodys principal means of delivering oxygen to body tissues via the blood. ... Viscosity is a measure of the resistance of a fluid to deform under shear stress. ...


Recently, in 2005, a mutation in the JAK2 kinase (V617F) was found by multiple research groups [11][12] to be strongly associated with polycythemia vera. JAK2 is a member of the Janus kinase family. This mutation may be helpful in making a diagnosis or as a target for future therapy. JAK2 (Janus kinase 2 (a protein tyrosine kinase)) is a human gene that encodes for a protein called Janus kinase 2, a tyrosine kinase of the non-receptor type, involved in interleukin 3 signal transduction. ... Janus kinase< (JAK) is a family of intracellular non-receptor tyrosine kinases, ranging from 120-140 kDa, that transduce cytokine-mediated signals via the JAK-STAT pathway. ...


As a consequence of the above, people with untreated PV are at a risk of various thrombotic events (deep venous thrombosis, pulmonary embolism), heart attack and stroke, and have a substantial risk of Budd-Chiari syndrome (hepatic vein thrombosis). The condition is considered chronic; no cure exists. Symptomatic treatment (see below) can normalize the blood count and most patients can live a normal life for years. Thrombosis is the formation of a clot or thrombus inside a blood vessel, obstructing the flow of blood through the circulatory system. ... It has been suggested that Deep Vein Thrombosis be merged into this article or section. ... Acute myocardial infarction (AMI or MI), more commonly known as a heart attack, is a disease state that occurs when the blood supply to a part of the heart is interrupted. ... Stroke (or cerebrovascular accident or CVA) is the clinical designation for a rapidly developing loss of brain function due to an interruption in the blood supply to all or part of the brain. ... In medicine (gastroenterology and hepatology), Budd-Chiari syndrome is the clinical picture caused by occlusion of the hepatic vein. ...


Treatment

As the condition cannot be cured, treatment focuses on treating symptoms and reducing thrombotic complications reducing the erythrocyte levels.


Bloodletting or phlebotomy is one form of treatment, which often may be combined with other therapies. The removal of blood from the body reduces the blood volume and brings down the hematocrit levels; in patients with polycythemia vera, this reduces the risk of blood clots. Phlebotomy is typically performed in people with polycythemia vera to bring their hematocrit (red blood cell percentage) down below 45 for men or 42 for women.[13] Ancient Greek painting in a vase, showing a physician (iatros) bleeding a patient. ... The hematocrit (Ht or HCT) and packed cell volume (PCV) are measures of the proportion of blood volume that is occupied by red blood cells. ...


Low dose aspirin is often prescribed. Research has shown that aspirin reduces the risk for various thrombotic complications. Aspirin, or acetylsalicylic acid (IPA: ), (acetosal) is a drug in the family of salicylates, often used as an analgesic (to relieve minor aches and pains), antipyretic (to reduce fever), and as an anti-inflammatory. ...


Chemotherapy for polycythemia may be used sparingly, when the rate of bloodlettings required to maintain normal hematocrit is not acceptable. This is usually with a "cytoreductive agent" (hydroxyurea, also known as hydroxycarbamide). Chemotherapy is the use of chemical substances to treat disease. ... Hydroxyurea chemical structure Hydroxyurea or hydroxycarbamide (rINN), (brand names include Hydrea®) is an antineoplastic drug used in hematological malignancies. ... Hydroxyurea or hydroxycarbamide (brand names include Hydrea®) is an antineoplastic drug used in hematological malignancies. ...


The tendency to avoid chemotherapy if possible, especially in young patients, is due to research indicating increased risk of transformation to AML, and while hydroxyurea is considered safer in this aspect, there is still some debate about its long-term safety. Acute myelogenous leukemia (AML), also known as acute myeloid leukemia, is a cancer of the myeloid line of blood cells. ...


In the past, injection of radioactive isotopes was used as another means to suppress the bone marrow. Such treatment is now avoided due to a high rate of AML transformation.


Other therapies include interferon injections, and in cases where secondary thrombocytosis (high platelet count) is present, anagrelide may be prescribed. Interferons (IFNs) are natural proteins produced by the cells of the immune system of most vertebrates in response to challenges by foreign agents such as viruses, bacteria, parasites and tumor cells. ... Thrombocytosis is the presence of high platelet counts in the blood, and can be either reactive or primary (also termed essential and caused by a myeloproliferative disease). ... A 250 ml bag of newly collected platelets. ... Anagrelide (Agrylin®/Xagrid®, Shire) is a drug used for the treatment of essential thrombocytosis (ET; essential thrombocythemia). ...


Bone marrow transplants are rarely undertaken in polycythemia patients - since this condition is non-fatal if treated and monitored, the benefits rarely outweigh the risks involved in such a procedure. Bone marrow transplantation or hematopoietic stem cell transplantation (HSCT) is a medical procedure in the field of hematology and oncology that involves transplantation of hematopoietic stem cells (HSC). ...


External links

References

  1. ^ Passamonti F, Malabarba L, Orlandi E, Baratè C, Canevari A, Brusamolino E, Bonfichi M, Arcaini L, Caberlon S, Pascutto C, Lazzarino M (2003). "Polycythemia vera in young patients: a study on the long-term risk of thrombosis, myelofibrosis and leukemia.". Haematologica 88 (1): 13-8. PMID 12551821. 
  2. ^ a b c Berlin, NI. (1975). "Diagnosis and classification of polycythemias". Semin Hematol 12: 339.
  3. ^ a b Anía B, Suman V, Sobell J, Codd M, Silverstein M, Melton L (1994). "Trends in the incidence of polycythemia vera among Olmsted County, Minnesota residents, 1935-1989.". Am J Hematol 47 (2): 89-93. PMID 8092146. 
  4. ^ Steinman H, Kobza-Black A, Lotti T, Brunetti L, Panconesi E, Greaves M (1987). "Polycythaemia rubra vera and water-induced pruritus: blood histamine levels and cutaneous fibrinolytic activity before and after water challenge.". Br J Dermatol 116 (3): 329-33. PMID 3567071. 
  5. ^ Jackson N, Burt D, Crocker J, Boughton B (1987). "Skin mast cells in polycythaemia vera: relationship to the pathogenesis and treatment of pruritus.". Br J Dermatol 116 (1): 21-9. PMID 3814512. 
  6. ^ Fjellner B, Hägermark O (1979). "Pruritus in polycythemia vera: treatment with aspirin and possibility of platelet involvement.". Acta Derm Venereol 59 (6): 505-12. PMID 94209. 
  7. ^ Torgano G, Mandelli C, Massaro P, Abbiati C, Ponzetto A, Bertinieri G, Bogetto S, Terruzzi E, de Franchis R (2002). "Gastroduodenal lesions in polycythaemia vera: frequency and role of Helicobacter pylori.". Br J Haematol 117 (1): 198-202. PMID 11918555. 
  8. ^ van Genderen P, Michiels J (1997). "Erythromelalgia: a pathognomonic microvascular thrombotic complication in essential thrombocythemia and polycythemia vera.". Semin Thromb Hemost 23 (4): 357-63. PMID 9263352. 
  9. ^ Michiels J (1997). "Erythromelalgia and vascular complications in polycythemia vera.". Semin Thromb Hemost 23 (5): 441-54. PMID 9387203. 
  10. ^ Landolfi R, Ciabattoni G, Patrignani P, Castellana M, Pogliani E, Bizzi B, Patrono C (1992). "Increased thromboxane biosynthesis in patients with polycythemia vera: evidence for aspirin-suppressible platelet activation in vivo.". Blood 80 (8): 1965-71. PMID 1327286. 
  11. ^ Baxter EJ, Scott LM, Campbell PJ, East C, Fourouclas N, Swanton S, Vassiliou GS, Bench AJ, Boyd EM, Curtin N, Scott MA, Erber WN, Green AR (2005). "Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders". Lancet 365 (9464): 1054-61. PMID 15781101. 
  12. ^ Levine RL, Wadleigh M, Cools J, Ebert BL, Wernig G, Huntly BJ, Boggon TJ, Wlodarska I, Clark JJ, Moore S, Adelsperger J, Koo S, Lee JC, Gabriel S, Mercher T, D'Andrea A, Frohling S, Dohner K, Marynen P, Vandenberghe P, Mesa RA, Tefferi A, Griffin JD, Eck MJ, Sellers WR, Meyerson M, Golub TR, Lee SJ, Gilliland DG (2005). "Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis". Cancer Cell 7 (4): 387-97. PMID 15837627. 
  13. ^ Streiff MB, Smith B, Spivak JL (2002). "The diagnosis and management of polycythemia vera in the era since the Polycythemia Vera Study Group: a survey of American Society of Hematology members' practice patterns". Blood 99 (4): 1144-9. PMID 11830459. 

  Results from FactBites:
 
Polycythemia - Wikipedia, the free encyclopedia (727 words)
Polycythemia is a condition in which there is a net increase in the total circulating erythrocyte (red blood cell) mass of the body.
In primary polycythemia there may be 8 to 9 million and occasionally 11 million erythrocytes per cubic millimeter of blood, and the hematocrit may be as high as 70 to 80%.
Relative polycythemia is an apparent rise of the erythrocyte level in the blood; however, the underlying cause is reduced blood plasma.
  More results at FactBites »

 
 

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