{"id":12059,"date":"2025-03-13T23:24:06","date_gmt":"2025-03-13T22:24:06","guid":{"rendered":"https:\/\/valintermed.com\/?p=12059"},"modified":"2025-03-13T23:24:06","modified_gmt":"2025-03-13T22:24:06","slug":"mieloproliferativnye-novoobrazovaniya-mpn","status":"publish","type":"post","link":"https:\/\/valintermed.com\/en\/medlibrary\/myeloproliferative-neoplasms-mpn\/","title":{"rendered":"Myeloproliferative neoplasms (MPN)"},"content":{"rendered":"<div class=\"fpm_start\"><\/div>\n<p>Myeloproliferative neoplasms (MPNs) are a group of hemato-oncological diseases characterized by abnormal proliferation of bone marrow cells. The main diseases in this group are polycythemia vera, essential thrombocythemia, and idiopathic myelofibrosis. These diseases are caused by mutations that lead to dysregulation of cell growth, which is manifested by an increase in the number of cells in the blood and their abnormal function. In MPNs, changes in blood composition are observed, including an increase in the number of red blood cells, white blood cells, and platelets, which entails the risk of thrombosis and, in some cases, progression to acute leukemia. Despite the presence of clinical manifestations and high morbidity, MPNs remain poorly understood in the context of pathogenesis and therapy.<\/p>\n<div id=\"ez-toc-container\" class=\"ez-toc-v2_0_85 counter-flat ez-toc-counter ez-toc-light-blue ez-toc-container-direction\">\n<div class=\"ez-toc-title-container\">\n<p class=\"ez-toc-title\" style=\"cursor:inherit\">Content<\/p>\n<span class=\"ez-toc-title-toggle\"><a href=\"#\" class=\"ez-toc-pull-right ez-toc-btn ez-toc-btn-xs ez-toc-btn-default ez-toc-toggle\" aria-label=\"Toggle Table of Content\"><span class=\"ez-toc-js-icon-con\"><span class=\"\"><span class=\"eztoc-hide\" style=\"display:none;\">Toggle<\/span><span class=\"ez-toc-icon-toggle-span\"><svg style=\"fill: #999;color:#999\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" class=\"list-377408\" width=\"20px\" height=\"20px\" viewbox=\"0 0 24 24\" fill=\"none\"><path d=\"M6 6H4v2h2V6zm14 0H8v2h12V6zM4 11h2v2H4v-2zm16 0H8v2h12v-2zM4 16h2v2H4v-2zm16 0H8v2h12v-2z\" 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href=\"https:\/\/valintermed.com\/en\/medlibrary\/myeloproliferative-neoplasms-mpn\/#%D0%98%D1%81%D1%82%D0%BE%D1%80%D0%B8%D1%8F_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D0%B8_%D0%B8%D0%BD%D1%82%D0%B5%D1%80%D0%B5%D1%81%D0%BD%D1%8B%D0%B5_%D0%B8%D1%81%D1%82%D0%BE%D1%80%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B8%D0%B5_%D1%84%D0%B0%D0%BA%D1%82%D1%8B\" >History of the disease and interesting historical facts<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-2\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/myeloproliferative-neoplasms-mpn\/#%D0%AD%D0%BF%D0%B8%D0%B4%D0%B5%D0%BC%D0%B8%D0%BE%D0%BB%D0%BE%D0%B3%D0%B8%D1%8F\" >Epidemiology<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-3\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/myeloproliferative-neoplasms-mpn\/#%D0%93%D0%B5%D0%BD%D0%B5%D1%82%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B0%D1%8F_%D0%BF%D1%80%D0%B5%D0%B4%D1%80%D0%B0%D1%81%D0%BF%D0%BE%D0%BB%D0%BE%D0%B6%D0%B5%D0%BD%D0%BD%D0%BE%D1%81%D1%82%D1%8C_%D0%BA_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%BC%D1%83_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8E\" >Genetic predisposition to this disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-4\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/myeloproliferative-neoplasms-mpn\/#%D0%A4%D0%B0%D0%BA%D1%82%D0%BE%D1%80%D1%8B_%D1%80%D0%B8%D1%81%D0%BA%D0%B0_%D0%B2%D0%BE%D0%B7%D0%BD%D0%B8%D0%BA%D0%BD%D0%BE%D0%B2%D0%B5%D0%BD%D0%B8%D1%8F_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >Risk factors for the development of this disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-5\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/myeloproliferative-neoplasms-mpn\/#%D0%94%D0%B8%D0%B0%D0%B3%D0%BD%D0%BE%D1%81%D1%82%D0%B8%D0%BA%D0%B0_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >Diagnosis of this disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-6\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/myeloproliferative-neoplasms-mpn\/#%D0%9B%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D0%B5\" >Treatment<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-7\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/myeloproliferative-neoplasms-mpn\/#%D0%A1%D0%BF%D0%B8%D1%81%D0%BE%D0%BA_%D0%BB%D0%B5%D0%BA%D0%B0%D1%80%D1%81%D1%82%D0%B2_%D0%BF%D1%80%D0%B8%D0%BC%D0%B5%D0%BD%D1%8F%D0%B5%D0%BC%D1%8B%D1%85_%D0%B4%D0%BB%D1%8F_%D0%BB%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D1%8F_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >List of medications used to treat this disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-8\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/myeloproliferative-neoplasms-mpn\/#%D0%9C%D0%BE%D0%BD%D0%B8%D1%82%D0%BE%D1%80%D0%B8%D0%BD%D0%B3_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >Disease monitoring<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-9\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/myeloproliferative-neoplasms-mpn\/#%D0%92%D0%BE%D0%B7%D1%80%D0%B0%D1%81%D1%82%D0%BD%D1%8B%D0%B5_%D0%BE%D1%81%D0%BE%D0%B1%D0%B5%D0%BD%D0%BD%D0%BE%D1%81%D1%82%D0%B8_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >Age-related features of the disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-10\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/myeloproliferative-neoplasms-mpn\/#%D0%92%D0%BE%D0%BF%D1%80%D0%BE%D1%81%D1%8B_%D0%B8_%D0%BE%D1%82%D0%B2%D0%B5%D1%82%D1%8B\" >Questions and Answers<\/a><\/li><\/ul><\/nav><\/div>\n<h2><span class=\"ez-toc-section\" id=\"%D0%98%D1%81%D1%82%D0%BE%D1%80%D0%B8%D1%8F_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D0%B8_%D0%B8%D0%BD%D1%82%D0%B5%D1%80%D0%B5%D1%81%D0%BD%D1%8B%D0%B5_%D0%B8%D1%81%D1%82%D0%BE%D1%80%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B8%D0%B5_%D1%84%D0%B0%D0%BA%D1%82%D1%8B\"><\/span>History of the disease and interesting historical facts<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Myeloproliferative neoplasms have a rich history dating back to the early 20th century. Polycythemia vera was originally described by physician and pathologist Walter Seligman in 1892. In the 1930s, the main clinical and morphological characteristics of these diseases were established. In the 1960s, the first ideas about genetic mutations playing a role in the pathogenesis of MPNs arose, when a link between the disease and chromosomal abnormalities was identified. In recent decades, significant progress has been made in understanding the molecular mechanisms leading to myeloproliferative neoplasms, in particular, the JAK2 gene mutation, discovered in 2005, has received wide recognition. This discovery was a turning point in the diagnosis and treatment of these diseases.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%AD%D0%BF%D0%B8%D0%B4%D0%B5%D0%BC%D0%B8%D0%BE%D0%BB%D0%BE%D0%B3%D0%B8%D1%8F\"><\/span>Epidemiology<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Epidemiological data indicate that MPNs are a relatively common group of diseases among people over 60 years of age. Polycythemia vera is reported to occur in approximately 2-3 cases per 100,000 people per year, while essential thrombocythemia is reported to occur in approximately 0.5-2 cases per 100,000 people per year. The overall incidence of MPNs is estimated to be 1 per 100,000 people, but this number may vary by region and ethnicity. Notably, men are more likely to develop these diseases than women, and the presence of chronic diseases such as veno-occlusive disease may contribute to an increased risk of developing MPNs.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%93%D0%B5%D0%BD%D0%B5%D1%82%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B0%D1%8F_%D0%BF%D1%80%D0%B5%D0%B4%D1%80%D0%B0%D1%81%D0%BF%D0%BE%D0%BB%D0%BE%D0%B6%D0%B5%D0%BD%D0%BD%D0%BE%D1%81%D1%82%D1%8C_%D0%BA_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%BC%D1%83_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8E\"><\/span>Genetic predisposition to this disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Modern studies have shown that genetic predisposition plays a key role in the development of myeloproliferative neoplasms. The most common genetic abnormality is the JAK2 V617F gene mutation, which is observed in approximately 95% patients with polycythemia vera and in 50-60% with essential thrombocythemia. In addition, mutations in the CALR and MPL gene segments have also been associated with this group of diseases. These mutations lead to the activation of signaling pathways that promote endless cell growth and division, which causes the clinical manifestations of MPN. The importance of molecular markers in the diagnosis and prognosis of the disease is steadily increasing, providing new opportunities for treatment and patient monitoring.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%A4%D0%B0%D0%BA%D1%82%D0%BE%D1%80%D1%8B_%D1%80%D0%B8%D1%81%D0%BA%D0%B0_%D0%B2%D0%BE%D0%B7%D0%BD%D0%B8%D0%BA%D0%BD%D0%BE%D0%B2%D0%B5%D0%BD%D0%B8%D1%8F_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>Risk factors for the development of this disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Risk factors for myeloproliferative neoplasms can be divided into several groups:<\/p>\n<ul>\n<li><strong>Physical factors:<\/strong> Radiation, including X-rays and radiation, increases the risk of developing MPN.<\/li>\n<li><strong>Chemical factors:<\/strong> Exposure to certain chemicals, such as benzene and some toxicants, is associated with an increased risk. <\/li>\n<li><strong>Viral infections:<\/strong> Some viruses, such as the Epstein-Barr virus, may contribute to the development of myeloproliferative diseases, but this mechanism is still being actively studied.<\/li>\n<li><strong>Chronic inflammatory diseases:<\/strong> Pathologies such as hepatitis and some autoimmune diseases may also increase the risk of developing these diseases.<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"%D0%94%D0%B8%D0%B0%D0%B3%D0%BD%D0%BE%D1%81%D1%82%D0%B8%D0%BA%D0%B0_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>Diagnosis of this disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Diagnostics of myeloproliferative neoplasms includes several key areas:<\/p>\n<ul>\n<li><strong>Main symptoms:<\/strong> Patients may experience headaches, itching, fatigue, night sweats, and signs of blood clots such as thrombosis or embolism.<\/li>\n<li><strong>Laboratory tests:<\/strong> Blood tests show increased levels of red blood cells, platelets, and white blood cells, and may also indicate the presence of specific genetic mutations.<\/li>\n<li><strong>Radiological examinations:<\/strong> Ultrasound may be used to evaluate the condition of the spleen and liver, and MRI or CT scan may be used for more detailed examination.<\/li>\n<li><strong>Other types of diagnostics:<\/strong> Bone marrow puncture allows for a morphological examination of cells and the detection of hyperplasia.<\/li>\n<li><strong>Differential diagnosis:<\/strong> It is important to exclude other diseases such as acute myeloid leukemia or reactive myeloproliferative states, which requires a comprehensive data analysis.<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"%D0%9B%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D0%B5\"><\/span>Treatment<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Treatment of myeloproliferative neoplasms depends on the type of disease, clinical stage and individual characteristics of the patient. <\/p>\n<ul>\n<li><strong>General treatment:<\/strong> Includes lifestyle changes, control of concomitant diseases and prevention of complications.<\/li>\n<li><strong>Pharmacological treatment:<\/strong> The mainstay of therapy is the use of antitumor drugs such as hydroxyurea and JAK2 inhibitors (eg, puxolitinib).<\/li>\n<li><strong>Surgical treatment:<\/strong> May be indicated in cases requiring removal of the spleen (splenectomy) due to severe splenomegaly or thrombus formation.<\/li>\n<li><strong>Other types of treatment:<\/strong> Blood and platelet transfusions to correct cytopenia and paleo-hemotherapy to control thrombus formation.<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"%D0%A1%D0%BF%D0%B8%D1%81%D0%BE%D0%BA_%D0%BB%D0%B5%D0%BA%D0%B0%D1%80%D1%81%D1%82%D0%B2_%D0%BF%D1%80%D0%B8%D0%BC%D0%B5%D0%BD%D1%8F%D0%B5%D0%BC%D1%8B%D1%85_%D0%B4%D0%BB%D1%8F_%D0%BB%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D1%8F_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>List of medications used to treat this disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Clinical practice includes the use of a number of drugs:<\/p>\n<ul>\n<li>Hydroxyurea<\/li>\n<li>Puxolitinib<\/li>\n<li>Tyrosine kinase inhibitors (eg, nilotinib)<\/li>\n<li>Low dose aspirin<\/li>\n<li>Erythropoietin for correction of anemia<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"%D0%9C%D0%BE%D0%BD%D0%B8%D1%82%D0%BE%D1%80%D0%B8%D0%BD%D0%B3_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>Disease monitoring<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Monitoring of myeloproliferative neoplasms includes regular follow-up examinations:<\/p><script data-noptimize=\"\" data-wpfc-render=\"false\">\nfpm_start( \"true\" );\n<\/script>\n\n<ul>\n<li><strong>Control stages:<\/strong> Blood tests and patient assessments are required every 3-6 months.<\/li>\n<li><strong>Forecast:<\/strong> The prognosis may vary depending on the type and stage of the disease and the presence of concomitant pathologies.<\/li>\n<li><strong>Complications:<\/strong> The most common complications include thrombosis, hemorrhagic manifestations and the possibility of transformation into acute myeloid leukemia.<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"%D0%92%D0%BE%D0%B7%D1%80%D0%B0%D1%81%D1%82%D0%BD%D1%8B%D0%B5_%D0%BE%D1%81%D0%BE%D0%B1%D0%B5%D0%BD%D0%BD%D0%BE%D1%81%D1%82%D0%B8_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>Age-related features of the disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>The course of myeloproliferative neoplasms in patients of different age groups may have its own characteristics:<\/p>\n<ul>\n<li><strong>Children:<\/strong> Although MPNs are rare in children, they may be less aggressive and have a better prognosis.<\/li>\n<li><strong>Young and middle aged:<\/strong> Diseases are most often diagnosed at the age of 40-60 years and usually occur with pronounced symptoms.<\/li>\n<li><strong>Elderly patients:<\/strong> In the elderly, the disease may occur with a large number of concomitant diseases, which significantly complicates treatment and requires a more individualized approach.<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"%D0%92%D0%BE%D0%BF%D1%80%D0%BE%D1%81%D1%8B_%D0%B8_%D0%BE%D1%82%D0%B2%D0%B5%D1%82%D1%8B\"><\/span>Questions and Answers<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<ul>\n<li><strong>What are myeloproliferative neoplasms?<\/strong> This is a group of diseases characterized by excessive production of blood cells in the bone marrow.<\/li>\n<li><strong>What are the main symptoms of MPN?<\/strong> The main symptoms include headache, fatigue, itching, thrombosis and splenomegaly.<\/li>\n<li><strong>How is MPN diagnosed?<\/strong> Diagnosis includes blood tests, bone marrow aspiration and genetic testing.<\/li>\n<li><strong>What medications are used to treat MPN?<\/strong> Hydroxyurea, JAK2 inhibitors and low dose aspirin are used.<\/li>\n<li><strong>What is the prognosis for patients with MPN?<\/strong> The prognosis depends on the type of disease and the presence of concomitant pathologies, but serious complications are possible.<\/li>\n<\/ul>\n<div class=\"fpm_end\"><\/div>","protected":false},"excerpt":{"rendered":"<p>Myeloproliferative neoplasms (MPN) are a group of hemato-oncological diseases characterized by abnormal proliferation of bone marrow cells. The main diseases in this group are<\/p>","protected":false},"author":1,"featured_media":20744,"comment_status":"open","ping_status":"","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[298],"tags":[],"class_list":["post-12059","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-medlibrary"],"_links":{"self":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts\/12059","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/comments?post=12059"}],"version-history":[{"count":1,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts\/12059\/revisions"}],"predecessor-version":[{"id":15061,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts\/12059\/revisions\/15061"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/media\/20744"}],"wp:attachment":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/media?parent=12059"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/categories?post=12059"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/tags?post=12059"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}