{"id":5230,"date":"2024-12-11T18:11:02","date_gmt":"2024-12-11T17:11:02","guid":{"rendered":"https:\/\/irccs.com\/patologie\/chronic-myeloproliferative-neoplasms\/"},"modified":"2026-03-26T11:44:23","modified_gmt":"2026-03-26T10:44:23","slug":"chronic-myeloproliferative-neoplasms","status":"publish","type":"pathology","link":"https:\/\/irccs.com\/en\/patologie\/chronic-myeloproliferative-neoplasms\/","title":{"rendered":"Chronic myeloproliferative neoplasms"},"content":{"rendered":"\n<h2 class=\"wp-block-heading\"><span  id=\"pathology\" class=\"h2_anchor\"><\/span>Pathology<\/h2>\n\n\n\n<p>Chronic myeloproliferative neoplasms are a <strong>group of blood diseases<\/strong> that share common features: they result from acquired genetic alterations that change the functioning of <strong>bone marrow stem cells<\/strong>, that is, those from which red blood cells, white blood cells, and platelets originate.<\/p>\n\n\n\n<p>Because of these mutations, cells continue to grow and multiply excessively. The result is an <strong>abnormal increase in one or more cell types in the blood<\/strong>, <strong>varying according to the disease subtype<\/strong>. At the level of the bone marrow, diseased cells can produce inflammatory changes with formation of fibrous tissue (bone marrow fibrosis). Sometimes, hematopoietic cells appear and reproduce in the spleen and liver (myeloid metaplasia).<\/p>\n\n\n\n<h3 class=\"wp-block-heading\">Types<\/h3>\n\n\n\n<p>According to the most recent international classifications (WHO 2022 and ICC 2022), chronic myeloproliferative neoplasms include:<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Chronic myeloid leukemia (CML)<\/strong><\/li>\n\n\n\n<li><strong>Polycythemia vera (PV)<\/strong><\/li>\n\n\n\n<li><strong>Essential thrombocythemia (TE)<\/strong><\/li>\n\n\n\n<li><strong>Myelofibrosis (MF)<\/strong>, which can appear from the beginning (primary) or evolve from PV or TE<\/li>\n<\/ul>\n\n\n\n<p>Alongside these more common forms, there are rarer entities, such as:<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>chronic neutrophilic leukemia (CNL<\/strong>)<\/li>\n\n\n\n<li><strong>chronic eosinophilic leukemia (CEL)<\/strong><\/li>\n\n\n\n<li><strong>juvenile myelomonocytic leukemia (JMML)<\/strong><\/li>\n\n\n\n<li><strong>chronic myeloproliferative neoplasms<\/strong>, not otherwise specified <strong>(MPN-NOS)<\/strong>, i.e., forms that do not fully fit the criteria of the previous ones but show the same biological features.<\/li>\n<\/ul>\n\n\n\n<h2 class=\"wp-block-heading\"><span  id=\"risk-factors\" class=\"h2_anchor\"><\/span>Risk factors<\/h2>\n\n\n\n<p>Chronic myeloproliferative neoplasms are rare diseases and <strong>often develop without known causes<\/strong>. Some factors that may predispose to an increased risk of developing them are:<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Age<\/strong>: They are more common in people over 60 years old, although they can occur at a younger age;<\/li>\n\n\n\n<li><strong>Gender<\/strong>: some forms, such as Polycythemia Vera, are slightly more common in men than in women;<\/li>\n\n\n\n<li><strong>Specific genetic mut<\/strong>ations: mutations such as <strong>JAK2, CALR or MPL<\/strong> are present in many patients and affect blood cell production. These mutations are not hereditary in almost all cases, but they can develop in the bone marrow throughout life;<\/li>\n\n\n\n<li><strong>Exposure to chemicals or radiation<\/strong>: long-term exposures to industrial chemicals or ionizing radiation can increase the risk, although in most cases there is no identifiable cause;<\/li>\n\n\n\n<li><strong>Family history<\/strong>: having relatives with a chronic myeloproliferative neoplasm may slightly increase the risk, but most patients do not have cases in the family.<\/li>\n<\/ul>\n\n\n\n<h2 class=\"wp-block-heading\"><span  id=\"diagnosis-and-examination\" class=\"h2_anchor\"><\/span>Diagnosis and examination<\/h2>\n\n\n\n<p>Chronic myeloproliferative neoplasms <strong>are generally chronic diseases<\/strong> with even a very long latency phase, so they can often be diagnosed occasionally.  <\/p>\n\n\n\n<h3 class=\"wp-block-heading\">Chronic myeloid leukemia (CML)<\/h3>\n\n\n\n<p>Chronic myeloid leukemia (CML) is a cancerous disease of hematopoietic stem cells characterized by<strong>increased proliferation of white blood cell precursors <\/strong>(&#8220;myeloid&#8221; series).  <\/p>\n\n\n\n<p>It is caused by a chromosomal alteration called a reciprocal translocation between chromosome 9 and chromosome 22 (i.e., a portion of chromosome 9 binds to chromosome 22 and vice versa) that generates the <strong>Philadelphia<\/strong> (Ph+) <strong>chromosome<\/strong>, so named because it was discovered in 1960 in Philadelphia. This translocation generates an abnormal BCR-ABL hybrid gene, which in turn gives rise to an abnormal protein, a tyrosine kinase, that causes cells to proliferate uncontrollably.  <\/p>\n\n\n\n<p>To date, <strong>the<\/strong> root cause of the disease <strong>is not known with certainty<\/strong>, although there is an increased risk with exposure to ionizing radiation or potentially carcinogenic chemicals.  <\/p>\n\n\n\t<div id=\"block_69c50e068118d\" class=\"accordion-block acf-block\">\n\t\t<div class=\"my-4 lg:my-6\">\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Epidemiology<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p><span style=\"font-weight: 400\">CML is a <\/span><b>rare disease that affects about 2 in 100,000 people each year.<\/b><span style=\"font-weight: 400\"> It is a disease that occurs mainly in old age: and about half of the cases are diagnosed in people aged 65 years or older, with a slight prevalence in men. <\/span> <\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Symptoms and clinical course<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p><span style=\"font-weight: 400\">Chronic myeloid leukemia generally presents in a chronic phase, typically asymptomatic or with <\/span><b>mild clinical symptoms <\/b><span style=\"font-weight: 400\">such as fever, marked fatigue (asthenia), and weight loss. <\/span> <\/p>\n<p><span style=\"font-weight: 400\">An<strong> enlargement of the spleen<\/strong> (splenomegaly) can also be found, which can be asymptomatic or, if important, determining postprandial sense of repletion, left flank pain, and increased volume of the abdomen.<\/span><\/p>\n<p><span style=\"font-weight: 400\">In a minority of cases, the disease occurs or evolves into a more advanced stage, characterized by an increase in immature cells that give rise to white blood cells, the so-called blasts. The advanced phase of CML is distinguished into accelerated phase or blastic crisis based on the number of blasts and other biological features, such as the number of platelets or the presence of other chromosomal abnormalities.<\/span><\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Diagnosis<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p><span style=\"font-weight: 400\">In almost all patients, the diagnosis is made during the chronic phase, often absolutely randomly through the <\/span><b>normal blood tests<\/b><span style=\"font-weight: 400\">, performed for routine or other causes. <\/span> <\/p>\n<p><span style=\"font-weight: 400\">The blood count shows an <\/span><b>increased number of white blood cells<\/b><span style=\"font-weight: 400\"> (leukocytosis) and\/or increased platelets (plateletosis or thrombocytosis), although in some cases platelets may be reduced (plateletopenia). <\/span> <\/p>\n<p><span style=\"font-weight: 400\">Confirmation of the diagnosis is through:  <\/span><\/p>\n<ul>\n<li style=\"font-weight: 400\"><b>blood tests<\/b><span style=\"font-weight: 400\">, to evaluate the CBC, LDH value, liver and kidney function, and uric acid assay; <\/span> <\/li>\n<li style=\"font-weight: 400\"><b>bone marrow analysis<\/b><span style=\"font-weight: 400\">o, which is the factory of red blood cells, white blood cells, and platelets, by means of a sampling (osteomidullary biopsy), which is usually performed under local anesthesia, at the level of the postero-superior iliac crest of the pelvis (at the top of the buttock). In such a procedure, bone marrow blood is aspirated and a small cylinder of bone is taken. The histological specimen is processed and analyzed at the pathology laboratory;<\/span><\/li>\n<li style=\"font-weight: 400\"><b>The microscopic analysis of peripheral and bone marrow blood smear<\/b><span style=\"font-weight: 400\"> to see cell morphology and count the number of immature abnormal cells (blasts); <\/span> <\/li>\n<li style=\"font-weight: 400\"><b>cytogenetic examination and a FISH (fluorescent in situ hybridization) on bone marrow blood <\/b><span style=\"font-weight: 400\">to study chromosomes and highlight the Ph+ chromosome, the presence of which is diagnostic for CML; <\/span> <\/li>\n<li style=\"font-weight: 400\"><b>The molecular analysis on peripheral blood<\/b><span style=\"font-weight: 400\"> which makes it possible to assess the presence and quantify the transcript of the BCR-ABL gene and thus residual leukemic cells, and is useful for monitoring response to therapy; <\/span> <\/li>\n<li style=\"font-weight: 400\"><b>chest X-ray, ultrasound and in selected cases CT scan of the abdomen<\/b><span style=\"font-weight: 400\">, useful for diagnostic completion. These are examinations that are performed at the Division of Radiodiagnostics.<\/span><\/li>\n<\/ul>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Therapy and response assessment<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p><span style=\"font-weight: 400\">CML therapy has been completely revolutionized by the advent of the <\/span><b>Tyrosine kinase inhibitors (TKIs)<\/b><span style=\"font-weight: 400\">, which ushered in the era of targeted therapies. These are specifically directed against the abnormal protein produced by the Ph+ chromosome.<\/span><\/p>\n<p><span style=\"font-weight: 400\">Imatinib was the progenitor of this family of drugs, but to date we have other 2nd, 3rd, and 4th generation tyrosine kinase inhibitors available, such as dasatinib, nilotinib, bosutinib, ponatinib, and asciminib, which act on the same target and are all taken orally.  <\/span><\/p>\n<p><span style=\"font-weight: 400\">The physician will choose the most appropriate therapy for each patient depending on the toxicity profile of each drug, the patient&#8217;s associated diseases, and the biological characteristics of the disease (e.g., the presence of BCR\/ABL mutations).  <\/span><\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Response monitoring<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p><span style=\"font-weight: 400\">The effectiveness of therapy is assessed <\/span><b>initially by examination of peripheral venous blood<\/b><span style=\"font-weight: 400\">, to verify the achievement of a complete hematologic response. This examination is repeated at intervals ranging from one week to three months to check toxicity and to monitor response.  <\/span><\/p>\n<p><span style=\"font-weight: 400\">Once a complete hematologic response is obtained, cytogenetics and molecular biology are used to determine residual disease levels at the 3rd, 6th, and 12th months of therapy and then at intervals ranging from 3 to 6 months.<\/span><\/p>\n<p><span style=\"font-weight: 400\">The goal of CML therapy is to achieve early a complete hematologic response, that is, normalization of CBC values, and later disappearance of the Philadelphia chromosome from bone marrow cells (complete cytogenetic response) and finally reduction to disappearance of the BCR-ABL transcript from peripheral blood (molecular response).  <\/span><\/p>\n<p><span style=\"font-weight: 400\">In patients who achieve a profound molecular response with a significant and stable reduction in leukemic cells as measured by BCR\/ABL transcript, discontinuation of the tyrosine kinase inhibitor can be attempted after a prolonged period of treatment (typically at least 5 years), with close clinical-laboratory monitoring.<\/span><\/p>\n<p><span style=\"font-weight: 400\">If there is no response to the TKI used in the first line or in case of toxicity, it is possible to replace the current drug with a different tyrosine kinase inhibitor that can overcome any resistance mechanisms of the leukemic cells. In cases where the response is still not achieved or the disease evolves into its acute phase, one treatment option may be stem cell transplantation.<\/span><\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t<\/div>\n\n\t<\/div>\n\n\n\n<h3 class=\"wp-block-heading\">Polycythemia vera (PV)<\/h3>\n\n\n\n<p>Polycythemia vera (PV) is a myeloproliferative disease characterized by <strong>uncontrolled production of red blood cells (erythrocytosis)<\/strong> and, in some cases, <strong>white blood cells and platelets<\/strong>.<\/p>\n\n\n\n<p>It is caused by mutation of the JAK2 gene at the stem cell level, resulting in continuous stimulation of blood cell production. It is usually diagnosed by the finding on the<strong>CBC<\/strong> of an increase in hemoglobin and hematocrit (percentage of red blood cells relative to the entire blood volume) values.<\/p>\n\n\n\t<div id=\"block_69c50e068122c\" class=\"accordion-block acf-block\">\n\t\t<div class=\"my-4 lg:my-6\">\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Epidemiology<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p><span style=\"font-weight: 400\">PV occurs in about <\/span><b>1-2 people per 100,000<\/b><span style=\"font-weight: 400\">. <\/span> <\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Symptoms and clinical course<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p><span style=\"font-weight: 400\">Patients with polycythemia vera <\/span><b>may be asymptomatic or present with symptoms related to increased blood viscosity<\/b><span style=\"font-weight: 400\">, such as headache, vision changes, itching after bathing or showering, redness and warmth of hands and feet, often accompanied by burning (erythromelalgia). The liver and spleen may become enlarged as both begin to produce blood cells.  <\/span><\/p>\n<p><span style=\"font-weight: 400\">In addition, Polycythemia Vera is characterized by an <\/span><b>increased risk of cerebral or cardiac ischemic events and venous thrombosis<\/b><span style=\"font-weight: 400\">, which can sometimes be the onset symptom of the disease.<\/span><\/p>\n<p><span style=\"font-weight: 400\">PV is a chronic disease that with current treatment strategies is associated with<strong> prolonged survival<\/strong>. In fact, only a minority of patients develop a more advanced form of myeloproliferative disease, such as myelofibrosis, over the years, and an even smaller percentage develop acute leukemia.<\/span><\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Diagnosis<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p><span style=\"font-weight: 400\">The diagnosis of Polycythemia Vera is usually made by the <\/span><b>finding on the CBC of elevated red blood cell, hemoglobin, and hematocrit values<\/b><span style=\"font-weight: 400\">, sometimes associated with increased platelet and white blood cell counts. <\/span> <\/p>\n<p><span style=\"font-weight: 400\">With a careful medical history (evaluation of family history and previous or concomitant diseases), forms of erythrocytosis caused by other diseases (secondary erythrocytosis) or the very rare congenital forms should be excluded.<\/span><\/p>\n<p><span style=\"font-weight: 400\">The main diagnostic tests are:<\/span><\/p>\n<ul>\n<li style=\"font-weight: 400\"><b>assay of blood levels of erythropoietin<\/b><span style=\"font-weight: 400\">, a hormone produced by the kidney that stimulates the bone marrow to produce red blood cells. Erythropoietin levels are usually below normal in PV, while they may be normal or elevated in secondary forms;<\/span><\/li>\n<li style=\"font-weight: 400\"><b>The search for mutations in the JAK2 gene on peripheral venous blood<\/b><span style=\"font-weight: 400\">, present in almost all patients; <\/span> <\/li>\n<li style=\"font-weight: 400\"><b>The search for other mutations associated with myeloproliferative syndromes<\/b><span style=\"font-weight: 400\"> such as BCR\/ABL, CALR, MPL<\/span><\/li>\n<li style=\"font-weight: 400\"><b>bone marrow analysis<\/b><span style=\"font-weight: 400\">, which is the factory of red blood cells, white blood cells and platelets, by means of a sampling (osteomidullary biopsy), which is usually performed under local anesthesia, at the level of the postero-superior iliac crest of the pelvis (at the top of the buttock). In such a procedure, bone marrow blood is aspirated and a small cylinder of bone is taken. The histological specimen is processed and analyzed at the pathology laboratory. Morphologically, in the bone marrow, hyperplasia of all three hematopoietic cell lines, red blood cells, white blood cells, and platelets (panmyeloses), is evident.<\/span><\/li>\n<\/ul>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Therapy<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p><span style=\"font-weight: 400\">The therapy of Polycythemia Vera has as its first goal. <\/span><b>to reduce the incidence of thrombotic events<\/b><span style=\"font-weight: 400\">.<\/span><\/p>\n<p><span style=\"font-weight: 400\">For this, the following are used <\/span><b>antiplatelet drugs<\/b><span style=\"font-weight: 400\">, such as acetyl salicylic acid or, in cases of previous deep vein thrombosis, anticoagulant drugs, such as warfarin or direct oral anticoagulants (NAOs). <\/span> <\/p>\n<p><span style=\"font-weight: 400\">To reduce the number of circulating red blood cells, the following can be performed <\/span><b>periodic bloodletting<\/b><span style=\"font-weight: 400\">i, which consist of taking about 400 cc of blood and reinfusing equal amounts of saline, with the goal of keeping the hematocrit below 45 percent. <\/span> <\/p>\n<p><span style=\"font-weight: 400\">In patients older than 60 years of age who have a history of thrombotic events or who fail to normalize blood counts with bloodletting alone (e.g., due to increased white blood cells and platelets), the following are used <\/span><b>drugs defined as &#8220;cytoreductive&#8221;<\/b><span style=\"font-weight: 400\">, that is, that lower blood cell values.<\/span><\/p>\n<p><span style=\"font-weight: 400\">The drug of first choice is usually hydroxyurea, which acts on cell replication by reducing it. It is taken chronically by mouth at a dosage that varies from patient to patient.<\/span><\/p>\n<p><span style=\"font-weight: 400\">If hydroxyurea therapy is not effective or is associated with major side effects, other drugs such as<strong>interferon<\/strong>, JAK2 inhibitors, or in elderly patients alkylating drugs (such as busulfan) may be used.<\/span><\/p>\n<p><span style=\"font-weight: 400\">Interferon is an immunomodulatory drug, which acts on the immune system by reducing the number of red blood cells, white blood cells, and platelets. Currently, extended-release (pegylated) interferon formulations are used, which are administered subcutaneously every 2 or more weeks depending on response (e.g., ro-peg-ineterferon alpha2b).<\/span><\/p>\n<p><span style=\"font-weight: 400\">In addition, in patients intolerant or unresponsive to hydroxyurea, ruxolitinib, a drug-target that acts by inhibiting the JAK2 signaling pathway, can be used.  <\/span><\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t<\/div>\n\n\t<\/div>\n\n\n\n<h3 class=\"wp-block-heading\">Essential thrombocythemia (TE)<\/h3>\n\n\n\n<p>Essential thrombocythemia (TE) is a myeloproliferative neoplasm <strong>characterized by excessive platelet production causing abnormalities in blood coagulation <\/strong>with the occurrence of thrombotic phenomena or, paradoxically in rare cases, bleeding.  <\/p>\n\n\n\t<div id=\"block_69c50e0681294\" class=\"accordion-block acf-block\">\n\t\t<div class=\"my-4 lg:my-6\">\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Epidemiology<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p><span style=\"font-weight: 400\">The incidence is estimated to be approx. <\/span><b>1.5-2.4 cases per 100,000 people<\/b><span style=\"font-weight: 400\">. The disease is more common in women, and the average age at diagnosis is around 60, although about 20% of patients are diagnosed before age 40.<\/span><\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Clinical course<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p><span style=\"font-weight: 400\">Essential Thrombocythemia may or may not be symptomatic. I  <\/span><b>symptoms<\/b><span style=\"font-weight: 400\">, if present, <\/span><b>may be related to alterations in blood circulation <\/b><span style=\"font-weight: 400\">or, in some cases, to ischemic or thrombotic events caused by the blockage of certain blood vessels by blood clots (thrombi). <\/span> <\/p>\n<p><span style=\"font-weight: 400\">The most common symptoms are <strong>headache<\/strong>, <strong>tingling<\/strong> and other abnormal sensations of the fingertips, hands, and feet, chest pain, loss of vision or vision of dots, weakness, dizziness, bleeding, usually mild (such as nosebleeds, ease of bruising, mild exudate from the gums, or bleeding in the digestive tract). Despite the increase in platelet numbers, hemorrhagic rather than thrombotic events may occur, related to the interference of platelets with von Willebrand factor, an essential coagulation protein. The spleen may increase in volume, but this event (splenomegaly) is not so common.<\/span><\/p>\n<p><span style=\"font-weight: 400\"><strong>Life expectancy is almost normal <\/strong>, and the course of the disease is usually benign. About 10% of cases may evolve into a more advanced form of disease such as myelofibrosis, and evolution to acute leukemia is very rare (1-3%).  <\/span><\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Diagnosis<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p><span style=\"font-weight: 400\">The diagnosis of Essential Thrombocythemia is made on the basis of symptoms and after noting the<\/span><b>increased platelets in the blood count<\/b><span style=\"font-weight: 400\"> and ruling out causes of reactive plateletosis (blood loss, trauma, infection, neoplasm, splenectomy). <\/span> <\/p>\n<p><span style=\"font-weight: 400\">The cause lies in a <\/span><b>genetic mutation<\/b><span style=\"font-weight: 400\">, usually affecting the JAK2 kinase genes, the thrombopoietin receptor (MPL), a growth factor for platelet production, or the CALR gene. Identifying mutation in one of these genes confirms the diagnosis of essential thrombocythemia, but the absence of mutation does not exclude it.  <\/span><\/p>\n<p><span style=\"font-weight: 400\">In order to distinguish it from other forms of myeloproliferative disease, it is also necessary to perform BCR\/ABl mutation detection and bone marrow examination by osteomedicular biopsy and histological examination.<\/span><\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Therapy<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p>Thrombotic risk is stratified by the IPSET (International Prognostic Score for Thrombosis in Essential Thrombocythemia) score, which takes into account four main factors: age over 60 years, history of thrombotic events, presence of cardiovascular risk factors (such as smoking, hypertension, diabetes), and the JAK2 V617F genetic mutation. Based on these factors, patients are divided into low-, medium-, or high-risk groups for thrombosis, thus helping the physician choose the most appropriate therapy to prevent complications.<\/p>\n<p>Low-risk (no-factor) patients generally do not require antiplatelet or anticoagulant therapy, unless individually indicated.<\/p>\n<p>In patients at intermediate or high risk (presence of one or more factors), low-dose acetyl salicylic acid is recommended to prevent thrombotic events or anticoagulants in case of previous thrombotic events. In addition, high-risk patients need cytoreductive therapy to normalize platelet counts.<\/p>\n<p>Hydroxyurea, anagrelide, or, in younger patients, interferon in its pegylated form can be used for this purpose.<\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t<\/div>\n\n\t<\/div>\n\n\n\n<h3 class=\"wp-block-heading\">Idiopathic myelofibrosis (MF)<\/h3>\n\n\n\n<p><strong>Myelofibrosis<\/strong> is a form of chronic myeloproliferative neoplasm in which bone marrow is progressively replaced by fibrous tissue.  <br>When it arises without a previous history of other blood diseases, it is called <strong>primary myelofibrosis<\/strong>; if, on the other hand, it results from the evolution of Polycythemia Vera or Essential Thrombocythemia, it is called <strong>secondary myelofibrosis<\/strong> <strong>post PV or TE.<\/strong><\/p>\n\n\n\t<div id=\"block_69c50e06812f6\" class=\"accordion-block acf-block\">\n\t\t<div class=\"my-4 lg:my-6\">\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Epidemiology<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p><span style=\"font-weight: 400\">The incidence of the disease is low (estimated at approx. <\/span><b>0.5-1.3 cases per 100,000 people<\/b><span style=\"font-weight: 400\">) and falls within the definition of a <strong>rare disease<\/strong>. The average age at diagnosis is around 65 years.<\/span><\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Symptoms and clinical course<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p><span style=\"font-weight: 400\">Idiopathic Myelofibrosis initially may be asymptomatic. Next, the following may appear  <\/span><b>symptoms related to increased spleen<\/b><span style=\"font-weight: 400\"> (splenomegaly), <\/span>decreased CBC values or so-called &#8220;constitutional symptoms,&#8221; such as weight loss, generalized malaise, fever, night sweats.<\/p>\n<p><span style=\"font-weight: 400\">Splenomegaly may cause sense of abdominal distension, postprandial stuffiness, left flank pain.  <\/span><\/p>\n<p><span style=\"font-weight: 400\">Fatigue, weakness, dyspnea on exertion may occur in case of <strong>anemia<\/strong>.  <\/span><\/p>\n<p>In case of altered platelet count, which can be either elevated or reduced, there may be thrombotic or hemorrhagic manifestations.<\/p>\n<p>If the number of white blood cells is reduced, the body is at risk of infection.<\/p>\n<p><strong data-start=\"117\" data-end=\"162\">Survival in primary myelofibrosis<\/strong> may vary: some patients have a slow and stable course, others may have a more rapid evolution.<\/p>\n<p><strong data-start=\"292\" data-end=\"323\">Risk stratification<\/strong> systems exist that, by considering clinical data, blood tests and genetic information, help physicians identify different prognostic groups and choose the most appropriate treatment for each patient.<\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Diagnosis<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p><span style=\"font-weight: 400\">Idiopathic Myelofibrosis should be suspected in patients with splenomegaly and changes in the blood count that can be either increase or decrease in white blood cells, red blood cells, and platelets. If pathology is suspected, the following investigations should be performed in addition to the CBC:<\/span><\/p>\n<ul>\n<li style=\"font-weight: 400\"><b>the peripheral blood smear<\/b><span style=\"font-weight: 400\">o To assess the morphology of red blood cells and determine the number of immature cells. In fact, red blood cells with alterations in shape (e.g., dacryocytes) or precursors of white blood cells (myeloblasts) or red blood cells (erythroblasts) can be found in circulation;<\/span><\/li>\n<li style=\"font-weight: 400\"><b>LDH dosage<\/b><span style=\"font-weight: 400\">, which often occurs increased;<\/span><\/li>\n<li style=\"font-weight: 400\"><b>osteomidullary biopsy<\/b><span style=\"font-weight: 400\"> for histological examination and determination of the degree of fibrosis;<\/span><\/li>\n<li style=\"font-weight: 400\"><b>The search for mutations in JAK2, CALR or MPL<\/b><span style=\"font-weight: 400\">;<\/span><\/li>\n<li style=\"font-weight: 400\"><b>The search for other mutations on peripheral venous or medullary blood  <\/b><span style=\"font-weight: 400\">through a larger panel of next-generation sequencing (NGS) to detect gene mutations associated with increased risk of leukemic transformation. This analysis may be useful for prognosis, especially for younger patients who are candidates for hematopoietic stem cell transplantation;<\/span><\/li>\n<li style=\"font-weight: 400\"><b>abdominal ultrasound<\/b><span style=\"font-weight: 400\"> or, if necessary, <\/span><b>CT scan of the abdomen<\/b><span style=\"font-weight: 400\"> to measure liver and spleen and exclude thrombotic manifestations of abdominal vessels <\/span> <\/li>\n<\/ul>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Therapy<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p><span style=\"font-weight: 400\">Therapy is <strong>aimed at controlling symptoms and complications<\/strong>. Some patients may be observed without treatment.<\/span><\/p>\n<p><span style=\"font-weight: 400\">Patients with systemic symptoms, increased spleen, white blood cells or platelets should receive cytoreductive therapy.<\/span><\/p>\n<p><span style=\"font-weight: 400\">Depending on age, concomitant diseases, general condition, and risk of disease progression as determined by the major prognostic scores (IPSS, DIPSS, DIPSS-plus, MYSEC, MIPPS), patients may receive either <\/span><b>therapy with hydroxyurea, or with drugs -target<\/b><span style=\"font-weight: 400\"> that inhibit the JAK2 signaling pathway (JAK2 inhibitors) by reducing symptoms and splenomegaly, or be referred to an allogeneic stem cell transplantation pathway.<\/span><\/p>\n<p>For intermediate-high risk MFI with increased spleen, JAk2 inhibitors (ruxolitinib, momelotinib or fedratinib) are the drugs of first choice. They are administered by mouth chronically and are able to reduce symptoms associated with myelofibrosis and splenomegaly in a good percentage of patients. Momelotinib can also improve anemia when present at diagnosis. If therapy with these drugs fails, patients should be evaluated for experimental therapies; in fact, new JAK2 inhibitors and drugs that act on other cellular mechanisms are under advanced study.<\/p>\n<p><span style=\"font-weight: 400\">Low-dose corticosteroids may be used in conjunction with the drugs listed above or when they have failed or are contraindicated to control symptoms; androgens, erythropoietin, and transfusions to correct anemia; splenic radiotherapy<\/span><span style=\"font-weight: 400\"> to try to reduce spleen size when JAK2 inhibitors have failed or are contraindicated.<\/span><\/p>\n<p><span style=\"font-weight: 400\">Patients with high-risk myelofibrosis with age &lt;70-75 years should be evaluated early for allogeneic transplant candidacy, and be referred to an oncohematologist experienced in cell therapy. The procedure involves first administering preparatory chemotherapy that destroys all bone marrow cells and then infusing the patient with healthy stem cells from the donor, which are responsible for repopulating the blood and marrow itself. The Candiolo Institute is equipped with a Transplant <a href=\"https:\/\/irccs.com\/en\/divisioni-cliniche\/hemopoietic-stem-cell-transplantation-and-cellular-therapies-center\/\">Center<\/a> and a transplantology program.<\/span><\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t<\/div>\n\n\t<\/div>\n\n\n\n<h2 class=\"wp-block-heading\"><span  id=\"ongoing-support\" class=\"h2_anchor\"><\/span>Ongoing support<\/h2>\n\n\n\n<p>At our institute, we guarantee <strong>constant support before, during and after treatment <\/strong>to accompany each patient throughout the entire course of treatment and recovery.<\/p>\n\n\n\t<div id=\"block_69c50e068134b\" class=\"accordion-block acf-block\">\n\t\t<div class=\"my-4 lg:my-6\">\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Management of side effects  <\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p>All cancer treatments involve <strong>side effects<\/strong> that impact the patient&#8217;s quality of life more or less severely. Treatments for chronic myeloproliferative syndromes also involve side effects, both physical and psychological, that can change the way people cope with daily life.<\/p>\n<p><!-- \/wp:paragraph --> <!-- wp:paragraph --><\/p>\n<p>At the Candiolo Institute, attention to the patient&#8217;s quality of life remains a priority throughout the entire course of treatment: the physicians and nurses of the multidisciplinary team are available to provide the patient with all the support needed to manage the various side effects, particularly through nutritional counseling, psychological support and pain therapy.<\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Direct line to specialists<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p><span style=\"font-weight: 400\">To ensure <\/span><b>timely and direct support<\/b><span style=\"font-weight: 400\"> and receive timely answers to concerns and questions, a dedicated support service is in place at the Candiolo Institute for all patients.<\/span><\/p>\n<p><span style=\"font-weight: 400\">From Monday to Friday, from 8 a.m. to 5 p.m., you can <\/span><b>contact the secretariat of the oncology day hospital<\/b><span style=\"font-weight: 400\"> at 011.993.3775, reporting the need for urgent consultation.<\/span><\/p>\n<p><span style=\"font-weight: 400\">The patient will be <\/span><b>quickly put in touch with his or her medical specialist<\/b><span style=\"font-weight: 400\">, to receive clear answers and immediate support.<\/span><\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Continuing and palliative care<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p><span style=\"font-weight: 400\">The cancer patient is a person with <\/span><b>complex needs<\/b><span style=\"font-weight: 400\"> that requires <\/span><b>multidisciplinary support<\/b><span style=\"font-weight: 400\"> not only for the cancer disease, but also for all related issues.<\/span><\/p>\n<p><span style=\"font-weight: 400\">At the Candiolo Institute, patients who need or require it have access to <\/span><b>specialists in different areas<\/b><span style=\"font-weight: 400\"> to receive nutritional support, physical therapy, pain therapy and management of other associated conditions.<\/span><\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Psychological support  <\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<h3><span style=\"font-weight: 400\">The impact of cancer in a person&#8217;s life also affects the psychological sphere: in fact, falling ill with cancer is always a<\/span><b> traumatic event that affects all dimensions of the person<\/b><span style=\"font-weight: 400\"> and can generate anxiety, fear, anger, depression.<\/span><\/h3>\n<h3><span style=\"font-weight: 400\">In our institute, alongside cutting-edge therapies, the treatment and care pathway always includes a <\/span><b>qualified psycho-oncological support <\/b><span style=\"font-weight: 400\">that helps the patient cope positively not only with treatment but also with the delicate phase of physical and psychological recovery.<\/span><\/h3>\n<p><span style=\"font-weight: 400\">It is also possible to participate in<\/span><b> psychological support groups <\/b><span style=\"font-weight: 400\">to engage with other people who have gone through or are going through the same experience.<\/span><\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Social work<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p><span style=\"font-weight: 400\">The Social Service Department of the Candiolo Institute conducts <\/span><b>information and orientation interviews to patients and their families<\/b><span style=\"font-weight: 400\"> on how to access services in the area and how to obtain welfare and social security benefits provided by law (disability, benefits for aids and prostheses, work leave, etc.).<\/span><\/p>\n<p><span style=\"font-weight: 400\">The service operates on Wednesdays and Fridays from 9 a.m. to 1 p.m. (phone: 011 9933059).<\/span><\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Follow up<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p><strong>In many cases,<\/strong> chronic myeloproliferative syndromes <strong>only need to be monitored over time<\/strong>, with no need to initiate active treatment.<\/p>\n<p>In each case, during the follow-up period, through a <strong>series of examinations and visits<\/strong>, the progress of the disease or the side effects of the therapies given and their effectiveness are monitored, and the patient&#8217;s functional recovery is assessed.<\/p>\n<p><!-- \/wp:paragraph --> <!-- wp:paragraph --><\/p>\n<p>Follow-up examinations are especially important to <strong>intercept any disease development early<\/strong>, so as to intervene early and appropriately. For the patient, they are also a valuable opportunity for dialogue with their medical specialist.<\/p>\n<p><!-- \/wp:paragraph --> <!-- wp:paragraph --><\/p>\n<p>The follow-up course is planned with<strong> different timing and modalities depending on the type of chronic myeloproliferative syndrome<\/strong>, the therapy performed, the response obtained, and the patient&#8217;s own characteristics.<\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t<\/div>\n\n\t<\/div>\n\n\n\n<h2 class=\"wp-block-heading\"><span  id=\"interdisciplinary-group\" class=\"h2_anchor\"><\/span>Interdisciplinary Group<\/h2>\n\n\n\n<p>Every cancer requires, in all phases of disease management, a<strong> multidisciplinary approach<\/strong> that at the Candiolo Institute is guaranteed by a team of different specialists, belonging to the various clinical and surgical departments of the Institute: this team is called GIC (Interdisciplinary Care Group). The GIC ensures that<strong> each patient is taken care of throughout the diagnostic-therapeutic process<\/strong>, including prescribing and booking examinations and communicating with the patient and his or her family members. The GIC defines and shares a <strong>personalized care pathway<\/strong> for each patient, based not only on the type and stage of the tumor, but also on the patient&#8217;s own characteristics. The goal is to ensure that he or she has the best outcome both oncologically and functionally and the maintenance of a good quality of life.The Group also<strong> works closely with researchers<\/strong> at the Institute to ensure that patients have rapid access to the latest research-produced innovations in screening, diagnosis and treatment.<\/p>\n\n\n\n<h2 class=\"wp-block-heading\"><span  id=\"clinical-divisions\" class=\"h2_anchor\"><\/span>Clinical divisions  <\/h2>\n\n\n\n<p>The diagnostic-therapeutic pathway of chronic myeloproliferative syndromes at Candiolo involves several clinical divisions, including:<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><a href=\"https:\/\/irccs.com\/en\/divisioni-cliniche\/medical-oncology\/\" type=\"medical-area\" id=\"5169\">Medical Oncology<\/a><\/li>\n\n\n\n<li><a href=\"https:\/\/irccs.com\/en\/divisioni-cliniche\/oncology-day-hospital\/\" type=\"medical-area\" id=\"5183\">Day Hospital<\/a><\/li>\n\n\n\n<li><a href=\"https:\/\/irccs.com\/en\/divisioni-cliniche\/laboratory-analysis\/\" type=\"medical-area\" id=\"5113\">Laboratory Analysis<\/a><\/li>\n\n\n\n<li><a href=\"https:\/\/irccs.com\/en\/divisioni-cliniche\/pathologic-anatomy\/\" type=\"medical-area\" id=\"5118\">Pathologic anatomy<\/a><\/li>\n<\/ul>\n\n\n\n<h2 class=\"wp-block-heading\"><span  id=\"clinical-studies\" class=\"h2_anchor\"><\/span>Clinical studies<\/h2>\n\n\n\n<p><strong>Clinical research<\/strong> focuses on optimizing available therapies, using all generations of drugs and molecular inhibitors in a combined manner, with the goal also of preventing possible treatment resistance, both in <strong>Chronic Myeloid Leukemia<\/strong> and in other <strong>myeloproliferative malignancies<\/strong>.<\/p>\n\n\n\n<p>For patients with Chronic Myeloid Leukemia who respond effectively to drugs, achieving a <strong>profound molecular response<\/strong>, numerous clinical trials have shown the possibility of <strong>discontinuing therapy in a good percentage of patients<\/strong>, thus transforming a previously chronic treatment into a &#8220;time definite&#8221; treatment.<\/p>\n\n\n\n<p>In <strong>polycythemia vera<\/strong>, new drugs with novel mechanism of action, such as molecules that reduce hematocrit by acting on iron metabolism, are currently under investigation.<\/p>\n\n\n\n<p>In <strong>essential thrombocythemia<\/strong>, other interferon formulations and monoclonal antibodies against calreticulin are under investigation.<\/p>\n\n\n\n<p>In <strong>myelofibrosis<\/strong>, several drugs are being tested that act on different mechanisms underlying the disease. These include <strong>new tyrosine kinase inhibitors<\/strong> (such as pacritinib), <strong>BET inhibitors<\/strong> (pelabresib) and <strong>BCL-2 inhibitors<\/strong> (navitoclax). The BET and BCL-2 proteins are involved in DNA transcription, proliferation, and survival of cancer cells: blocking their activity allows the elimination of diseased cells.<\/p>\n\n\n\n<p>Other investigational drugs include <strong>MDM2 inhibitors<\/strong> (navtemadlin), which promote<strong>tumor cell self-elimination<\/strong> (apoptosis), and lend themselves to combination with JAK inhibitors through complementary mechanisms. <strong>BTK inhibitors<\/strong>, on the other hand, act on proteins involved in the inflammatory response, effectively reducing inflammation and being useful in patients with major inflammatory symptoms and <strong>splenomegaly<\/strong> (enlarged spleen).<\/p>\n\n\n\n<p>Researchers at the<strong>Candiolo Institute<\/strong> are actively engaged in several projects on chronic myeloproliferative neoplasms, following national and international guidelines and participating in <strong>experimental clinical trials<\/strong>.<\/p>\n\n\n\n<p>The institute is also a <strong>qualified center<\/strong> for <strong>integrated diagnostics of myeloid malignancies<\/strong>, enabling it to perform the most complex molecular analyses and offer patients the best diagnostic and therapeutic opportunities.<br>A major goal of the Institute is to collaborate with as many researchers as possible, providing sufficient data to draw objective conclusions about the effectiveness of therapeutic strategies in chronic myeloproliferative neoplasms.<\/p>\n\n\n\n<h2 class=\"wp-block-heading\"><span  id=\"why-choose-us\" class=\"h2_anchor\"><\/span><strong>Why choose us<\/strong><\/h2>\n\n\n\n<p>At the Candiolo IRCCS Institute, every patient with myeloproliferative syndromes is<strong> followed according to highly specialized standards, <\/strong>thanks to the synergistic work of a dedicated Interdisciplinary Care Group (ICG).<\/p>\n\n\n\t<div id=\"block_69c50e068138e\" class=\"accordion-block acf-block\">\n\t\t<div class=\"my-4 lg:my-6\">\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Clinical experience and tailored approach<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p>Due to the high number of cases treated each year, the Candiolo Institute is a <strong>national reference for taking care of esophageal cancer.<\/strong> Our experience enables us to deal with even the most complex situations, always with a <strong>personalized approach<\/strong> built on the clinical and personal profile of each patient.<\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Imaging technologies and advanced diagnostics<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p><!-- wp:paragraph -->Establishing the treatment plan always starts with an <strong>accurate and timely diagnosis<\/strong>. Patients have access to <strong>state-of-the-art imaging technologies<\/strong> that allow accurate assessment of the extent of the disease.<\/p>\n<p><!-- \/wp:paragraph --><\/p>\n<p><!-- wp:paragraph -->In addition, the Institute offers <strong>advanced and sophisticated laboratory investigations<\/strong>, including molecular and genomic analyses, which are critical for identifying biological features of cancer and guiding therapeutic decisions.<\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Minimally invasive surgical techniques and multidisciplinarity<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p>When indicated, surgery is performed with <strong>minimally invasive techniques<\/strong> (laparoscopic or thoracoscopic), which reduce operative trauma, promote faster recovery, and improve postoperative quality of life. Every treatment choice is defined within the GIC, ensuring a consistent and integrated approach.<\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Clinical research and access to trials<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<h3 class=\"wp-block-heading\"><\/h3>\n<p><!-- \/wp:heading --><\/p>\n<p><!-- wp:paragraph -->As an IRCCS, the Candiolo Institute combines clinical practice with a <strong>strong vocation for scientific research.<\/strong> Patients can be evaluated for inclusion in active <strong>clinical trials<\/strong>, which provide a real opportunity to access <strong>innovative therapies<\/strong> not yet available in standard practice. Collaboration between care and research is a distinctive value that translates into <strong>concrete opportunities for the patient<\/strong>.<\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t<div class=\"accordion group border-b border-white\/5\">\n\t\t\t<div class=\"flex items-center cursor-pointer group-hover:text-blue-light group-[.accordion--open]:text-red transition-colors\">\n\t\t\t\t<div class=\"rotate-90 text-blue-lighter group-[.accordion--open]:rotate-180 group-[.accordion--open]:text-red transition-all\">\n\t\t\t\t\t<svg width=\"18\" viewbox=\"0 0 500 500\" fill=\"currentColor\">\n\t\t\t\t\t\t<polygon points=\"250,60 100,400 400,400\"\/>\n\t\t\t\t\t<\/svg>\n\t\t\t\t<\/div>\n\t\t\t\t<h3 class=\"font-bold ml-5\">Care and support every step of the way<\/h3>\n\t\t\t<\/div>\n\t\t\t<div class=\"accordion-content max-h-0 overflow-hidden transition-all\">\n\t\t\t\t<div class=\"pb-5 pl-[38px] pt-1\">\n\t\t\t\t\t<p>The Interdisciplinary Care Group takes care of the person at every stage: from diagnosis to treatment to <strong>follow-up<\/strong>, with attention to <strong>nutritional support<\/strong>, <strong>psychological health<\/strong>, and <strong>reintegration into daily life<\/strong>. The organization of checkups, visits and treatment is designed to ensure <strong>continuity and serenity<\/strong>, always valuing the human dimension of care.<\/p>\n\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t<\/div>\n\n\t<\/div>\n","protected":false},"featured_media":0,"template":"","meta":{"_acf_changed":false,"footnotes":""},"class_list":["post-5230","pathology","type-pathology","status-publish","hentry"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.2 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Chronic myeloproliferative neoplasms - Istituto di Candiolo<\/title>\n<meta name=\"description\" content=\"At Candiolo IRCCS (Turin), we offer molecular diagnostics, personalized therapies and opportunities to participate in innovative clinical trials.\" \/>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/irccs.com\/en\/patologie\/chronic-myeloproliferative-neoplasms\/\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Chronic myeloproliferative neoplasms - 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