{"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":"2026-07-03T17:22:17","modified_gmt":"2026-07-03T15:22:17","slug":"chronic-myeloproliferative-neoplasms","status":"publish","type":"pathology","link":"https:\/\/inoc.it\/en\/pathology\/chronic-myeloproliferative-neoplasms\/","title":{"rendered":"Chronic Myeloproliferative Neoplasms (MPNs)"},"content":{"rendered":"\n<h2 class=\"wp-block-heading\"><span  id=\"pathology\" class=\"h2_anchor\"><\/span>Pathology<\/h2>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>Chronic myeloproliferative neoplasms (MPNs)<\/strong> are a group of blood disorders that share common biological features. They arise from acquired genetic alterations that affect bone marrow stem cells, which are responsible for the production of red blood cells, white blood cells, and platelets.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">As a result of these mutations, blood cells continue to grow and multiply excessively. This leads to an abnormal increase in one or more blood cell types, depending on the disease subtype. Within the bone marrow, abnormal cells may induce inflammatory changes and the formation of fibrous tissue (<strong>bone marrow fibrosis<\/strong>). In some cases, blood cell production may also occur in the spleen and liver, a process known as <strong>myeloid metaplasia<\/strong>.<\/p>\n\n\n\n<h3 class=\"wp-block-heading\">Types<\/h3>\n\n\n\n<p class=\"wp-block-paragraph\">According to the most recent international classifications (<strong>WHO 2022<\/strong> and <strong>ICC 2022<\/strong>), 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 (ET)<\/strong><\/li>\n\n\n\n<li><strong>Myelofibrosis (MF)<\/strong>, which may be present at diagnosis (<strong>primary myelofibrosis<\/strong>) or may develop from PV or ET<\/li>\n<\/ul>\n\n\n\n<p class=\"wp-block-paragraph\">In addition to these more common forms, there are rarer entities, including:<\/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>Myeloproliferative neoplasm, not otherwise specified (MPN NOS)<\/strong>, which includes forms that do not fully meet the diagnostic criteria for the conditions listed above but share similar biological characteristics.<\/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 class=\"wp-block-paragraph\">Chronic myeloproliferative neoplasms are rare diseases and often develop<strong> without an identifiable cause<\/strong>. Factors that may be associated with an increased risk include:<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Age:<\/strong> MPNs are more common in individuals over 60 years of age, although they can occur at younger ages.<\/li>\n\n\n\n<li><strong>Sex:<\/strong> Some forms, such as <strong>polycythemia vera<\/strong>, are slightly more common in men.<\/li>\n\n\n\n<li><strong>Specific genetic mutations:<\/strong> Mutations involving <strong>JAK2, CALR,<\/strong> or <strong>MPL<\/strong> are present in many patients and affect blood cell production. In almost all cases, these mutations are acquired rather than inherited and develop in the bone marrow during life.<\/li>\n\n\n\n<li><strong>Exposure to chemicals or radiation:<\/strong> Long term exposure to industrial chemicals or ionizing radiation may increase risk, although no identifiable cause is found in most patients.<\/li>\n\n\n\n<li><strong>Family history:<\/strong> Having relatives with a chronic myeloproliferative neoplasm may slightly increase risk, although most patients have no family history of the disease.<\/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 class=\"wp-block-paragraph\">Chronic myeloproliferative neoplasms are <strong>generally chronic diseases<\/strong> with a long asymptomatic phase. As a result, they are often diagnosed incidentally during routine blood testing.<\/p>\n\n\n\n<h3 class=\"wp-block-heading\"><strong>Chronic Myeloid Leukemia (CML)<\/strong><\/h3>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>Chronic myeloid leukemia (CML)<\/strong> is a malignant disorder of hematopoietic stem cells characterized by excessive proliferation of white blood cell precursors belonging to the myeloid lineage.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">The disease is caused by a chromosomal abnormality known as a <strong>reciprocal translocation between chromosomes 9 and 22<\/strong>, which generates the <strong>Philadelphia chromosome (Ph+)<\/strong>. This abnormality was first identified in Philadelphia in 1960. The translocation creates the <strong>BCR::ABL1 fusion gene<\/strong>, which produces an abnormal tyrosine kinase protein that drives uncontrolled cell proliferation.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">The underlying cause of this genetic alteration remains unknown, although exposure to ionizing radiation and certain potentially carcinogenic chemicals has been associated with an increased risk.<\/p>\n\n\n\t<div id=\"block_6a47d3a7568ba\" 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><b>Chronic myeloid leukemia (CML)<\/b><span style=\"font-weight: 400;\"> is a rare disease, with an annual incidence of approximately <\/span><b>2 cases per 100,000 people<\/b><span style=\"font-weight: 400;\">. It occurs predominantly in older adults, with about half of all diagnoses made in individuals aged <\/span><b>65 years or older<\/b><span style=\"font-weight: 400;\">. The disease is slightly more common in men than in women. <\/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><b>Chronic myeloid leukemia (CML)<\/b><span style=\"font-weight: 400;\"> most commonly presents in the <\/span><b>chronic phase<\/b><span style=\"font-weight: 400;\">, which is often asymptomatic or associated with mild symptoms such as <\/span><b>fever<\/b><span style=\"font-weight: 400;\">, <\/span><b>marked fatigue (asthenia)<\/b><span style=\"font-weight: 400;\">, and <\/span><b>weight loss<\/b><span style=\"font-weight: 400;\">.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">Patients may also develop <\/span><b>splenomegaly<\/b><span style=\"font-weight: 400;\"> (enlargement of the spleen), which can be asymptomatic or, when significant, may cause <\/span><b>early satiety after meals<\/b><span style=\"font-weight: 400;\">, <\/span><b>pain or discomfort in the left upper abdomen<\/b><span style=\"font-weight: 400;\">, and <\/span><b>abdominal distension<\/b><span style=\"font-weight: 400;\">.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">In a minority of cases, the disease presents in or progresses to a more advanced stage characterized by an increased number of <\/span><b>blasts<\/b><span style=\"font-weight: 400;\">, the immature precursor cells of white blood cells.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">The advanced stages of CML are classified as <\/span><b>accelerated phase<\/b><span style=\"font-weight: 400;\"> or <\/span><b>blast crisis<\/b><span style=\"font-weight: 400;\">, based on the percentage of blasts and other biological features, including platelet counts and the presence of additional 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 the vast majority of patients, <\/span><b>chronic myeloid leukemia (CML)<\/b><span style=\"font-weight: 400;\"> is diagnosed during the <\/span><b>chronic phase<\/b><span style=\"font-weight: 400;\">, often incidentally following routine blood tests performed for unrelated reasons.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">A <\/span><b>complete blood count (CBC)<\/b><span style=\"font-weight: 400;\"> typically shows an increased number of <\/span><b>white blood cells (leukocytosis)<\/b><span style=\"font-weight: 400;\"> and, in many cases, an elevated platelet count (<\/span><b>thrombocytosis<\/b><span style=\"font-weight: 400;\">). Less commonly, platelet levels may be reduced (<\/span><b>thrombocytopenia<\/b><span style=\"font-weight: 400;\">).<\/span><\/p>\n<p><span style=\"font-weight: 400;\">Confirmation of the diagnosis requires a combination of laboratory and bone marrow investigations, including:<\/span><\/p>\n<ul>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><b>Blood tests<\/b><span style=\"font-weight: 400;\"> to evaluate the CBC, <\/span><b>lactate dehydrogenase (LDH)<\/b><span style=\"font-weight: 400;\"> levels, liver and kidney function, and <\/span><b>uric acid<\/b><span style=\"font-weight: 400;\"> levels.<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><b>Bone marrow aspiration and bone marrow biopsy<\/b><span style=\"font-weight: 400;\">, performed to examine the tissue responsible for the production of red blood cells, white blood cells, and platelets. The procedure is usually carried out under local anesthesia at the level of the <\/span><b>posterior superior iliac crest<\/b><span style=\"font-weight: 400;\">. Bone marrow cells are aspirated, and a small core of bone tissue is collected for histological analysis by the pathology laboratory.<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><b>Microscopic examination of peripheral blood and bone marrow smears<\/b><span style=\"font-weight: 400;\"> to assess cell morphology and determine the proportion of immature abnormal cells (<\/span><b>blasts<\/b><span style=\"font-weight: 400;\">).<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><b>Cytogenetic analysis<\/b><span style=\"font-weight: 400;\"> and <\/span><b>fluorescence in situ hybridization (FISH)<\/b><span style=\"font-weight: 400;\"> on bone marrow samples to identify chromosomal abnormalities, including the <\/span><b>Philadelphia chromosome (Ph+)<\/b><span style=\"font-weight: 400;\">, whose presence is diagnostic of CML.<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><b>Molecular analysis of peripheral blood<\/b><span style=\"font-weight: 400;\"> to detect and quantify the <\/span><b>BCR::ABL1 transcript<\/b><span style=\"font-weight: 400;\">. This test is essential both for confirming the diagnosis and for monitoring response to treatment by measuring residual leukemic disease.<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><b>Imaging studies<\/b><span style=\"font-weight: 400;\">, including <\/span><b>chest X ray<\/b><span style=\"font-weight: 400;\">, <\/span><b>ultrasound<\/b><span style=\"font-weight: 400;\">, and, in selected cases, <\/span><b>abdominal CT scan<\/b><span style=\"font-weight: 400;\">, which may be used to complete the diagnostic assessment.<\/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;\">The treatment of <\/span><b>chronic myeloid leukemia (CML)<\/b><span style=\"font-weight: 400;\"> has been transformed by the introduction of <\/span><b>tyrosine kinase inhibitors (TKIs)<\/b><span style=\"font-weight: 400;\">, which marked the beginning of the era of <\/span><b>targeted therapies<\/b><span style=\"font-weight: 400;\">. These drugs specifically inhibit the abnormal protein produced by the <\/span><b>BCR\/ABL fusion gene<\/b><span style=\"font-weight: 400;\"> associated with the <\/span><b>Philadelphia chromosome (Ph+)<\/b><span style=\"font-weight: 400;\">.<\/span><\/p>\n<p><b>Imatinib<\/b><span style=\"font-weight: 400;\"> was the first TKI introduced into clinical practice. Since then, several additional agents have become available, including <\/span><b>dasatinib, nilotinib, bosutinib, ponatinib<\/b><span style=\"font-weight: 400;\">, and <\/span><b>asciminib<\/b><span style=\"font-weight: 400;\">. These therapies target the same molecular pathway and are administered orally.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">The choice of treatment is individualized and depends on several factors, including the <\/span><b>safety profile of each drug<\/b><span style=\"font-weight: 400;\">, the patient&#8217;s <\/span><b>comorbidities<\/b><span style=\"font-weight: 400;\">, and the <\/span><b>biological characteristics of the disease<\/b><span style=\"font-weight: 400;\">, such as the presence of specific <\/span><b>BCR\/ABL mutations<\/b><span style=\"font-weight: 400;\">.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">Assessment of treatment response is based on clinical, hematological, cytogenetic, and molecular parameters, allowing physicians to evaluate treatment effectiveness and guide ongoing management.<\/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 treatment is initially assessed through <\/span><b>peripheral blood tests<\/b><span style=\"font-weight: 400;\"> to confirm the achievement of a <\/span><b>complete hematologic response<\/b><span style=\"font-weight: 400;\">. These evaluations are repeated at intervals ranging from <\/span><b>one week to three months<\/b><span style=\"font-weight: 400;\"> to monitor both treatment response and potential toxicity.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">Once a complete hematologic response has been achieved, <\/span><b>cytogenetic<\/b><span style=\"font-weight: 400;\"> and <\/span><b>molecular assessments<\/b><span style=\"font-weight: 400;\"> are used to evaluate residual disease at <\/span><b>3, 6, and 12 months<\/b><span style=\"font-weight: 400;\"> after treatment initiation and subsequently every <\/span><b>3 to 6 months<\/b><span style=\"font-weight: 400;\">.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">The primary goals of treatment for <\/span><b>chronic myeloid leukemia (CML)<\/b><span style=\"font-weight: 400;\"> are:<\/span><\/p>\n<ul>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><span style=\"font-weight: 400;\">Achievement of a <\/span><b>complete hematologic response<\/b><span style=\"font-weight: 400;\">, with normalization of blood counts.<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><span style=\"font-weight: 400;\">Achievement of a <\/span><b>complete cytogenetic response<\/b><span style=\"font-weight: 400;\">, with disappearance of the <\/span><b>Philadelphia chromosome (Ph+)<\/b><span style=\"font-weight: 400;\"> from bone marrow cells.<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><span style=\"font-weight: 400;\">Achievement of a <\/span><b>molecular response<\/b><span style=\"font-weight: 400;\">, with a marked reduction or disappearance of the <\/span><b>BCR\/ABL1 transcript<\/b><span style=\"font-weight: 400;\"> in peripheral blood.<\/span><\/li>\n<\/ul>\n<p><span style=\"font-weight: 400;\">In patients who achieve a <\/span><b>deep and stable molecular response<\/b><span style=\"font-weight: 400;\">, demonstrated by a sustained reduction in residual leukemic cells, discontinuation of <\/span><b>tyrosine kinase inhibitor (TKI)<\/b><span style=\"font-weight: 400;\"> therapy may be considered after a prolonged treatment period, typically <\/span><b>at least five years<\/b><span style=\"font-weight: 400;\">, with careful clinical and laboratory monitoring.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">If the initial TKI fails to produce an adequate response or causes unacceptable toxicity, treatment can be switched to an alternative TKI capable of overcoming potential resistance mechanisms. In patients who do not achieve a satisfactory response despite these strategies, or whose disease progresses to an advanced phase, <\/span><b>allogeneic stem cell transplantation<\/b><span style=\"font-weight: 400;\"> may be considered as a treatment option.<\/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 class=\"wp-block-paragraph\"><strong>Polycythemia vera (PV)<\/strong> is a myeloproliferative neoplasm characterized by uncontrolled production of red blood cells (<strong>erythrocytosis<\/strong>) and, in some cases, increased production of white blood cells and platelets.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">The disease is caused by a mutation in the <strong>JAK2<\/strong> gene occurring at the stem cell level, resulting in continuous stimulation of blood cell production. It is often diagnosed following the detection of elevated <strong>hemoglobin<\/strong> and <strong>hematocrit<\/strong> levels on a complete blood count.<\/p>\n\n\n\t<div id=\"block_6a47d3a7569fd\" 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><b>Polycythemia vera (PV)<\/b><span style=\"font-weight: 400;\"> occurs in approximately <\/span><b>1 to 2 cases per 100,000 people<\/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 <\/span><b>polycythemia vera (PV)<\/b> <b>may be asymptomatic<\/b><span style=\"font-weight: 400;\"> or present symptoms related to increased <\/span><b>blood viscosity<\/b><span style=\"font-weight: 400;\">, including <\/span><b>headache<\/b><span style=\"font-weight: 400;\">, <\/span><b>visual disturbances<\/b><span style=\"font-weight: 400;\">, <\/span><b>pruritus after bathing or showering<\/b><span style=\"font-weight: 400;\">, and <\/span><b>redness, warmth, and burning of the hands and feet (erythromelalgia)<\/b><span style=\"font-weight: 400;\">.<\/span><\/p>\n<p><b>Hepatosplenomegaly<\/b><span style=\"font-weight: 400;\"> may also occur, as the liver and spleen may contribute to blood cell production.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">In addition, <\/span><b>polycythemia vera<\/b><span style=\"font-weight: 400;\"> is associated with an increased risk of <\/span><b>arterial and venous thrombotic events<\/b><span style=\"font-weight: 400;\">, including <\/span><b>cerebral and cardiac ischemia<\/b><span style=\"font-weight: 400;\">. In some cases, thrombosis may represent the first clinical manifestation of the disease.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">PV is a <\/span><b>chronic condition<\/b><span style=\"font-weight: 400;\"> that, with current treatment strategies, is associated with <\/span><b>prolonged survival<\/b><span style=\"font-weight: 400;\">. Only a minority of patients progress over time to a more advanced myeloproliferative disease such as <\/span><b>myelofibrosis<\/b><span style=\"font-weight: 400;\">, and an even smaller proportion develop <\/span><b>acute leukemia<\/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\">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 <\/span><b>polycythemia vera (PV)<\/b><span style=\"font-weight: 400;\"> is typically suggested by <\/span><b>complete blood count (CBC)<\/b><span style=\"font-weight: 400;\"> findings showing increased levels of <\/span><b>red blood cells, hemoglobin, and hematocrit<\/b><span style=\"font-weight: 400;\">, sometimes accompanied by elevated <\/span><b>platelet and white blood cell counts<\/b><span style=\"font-weight: 400;\">.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">A careful <\/span><b>clinical history<\/b><span style=\"font-weight: 400;\">, including evaluation of family history and previous or concurrent diseases, is essential to exclude other causes of erythrocytosis, such as <\/span><b>secondary erythrocytosis<\/b><span style=\"font-weight: 400;\"> or rare <\/span><b>congenital forms<\/b><span style=\"font-weight: 400;\">.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">The main diagnostic tests include:<\/span><\/p>\n<ul>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><b>Serum erythropoietin (EPO) levels<\/b><span style=\"font-weight: 400;\">, a hormone produced by the kidney that stimulates red blood cell production. In PV, EPO levels are typically <\/span><b>below normal<\/b><span style=\"font-weight: 400;\">, while they may be normal or elevated in secondary erythrocytosis.<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><b>Molecular testing for JAK2 mutations<\/b><span style=\"font-weight: 400;\"> on peripheral blood, which are present in almost all patients with PV.<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><b>Testing for additional mutations<\/b><span style=\"font-weight: 400;\"> associated with myeloproliferative neoplasms, including <\/span><b>BCR::ABL1, CALR, and MPL<\/b><span style=\"font-weight: 400;\">.<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><b>Bone marrow aspiration and biopsy<\/b><span style=\"font-weight: 400;\">, performed under local anesthesia at the level of the <\/span><b>posterior superior iliac crest<\/b><span style=\"font-weight: 400;\">. The procedure involves aspiration of bone marrow and collection of a small bone core, which is then processed for histological examination. In PV, the bone marrow typically shows <\/span><b>panmyelosis<\/b><span style=\"font-weight: 400;\">, characterized by hyperplasia of all three hematopoietic lineages: red blood cells, white blood cells, and platelets.<\/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 primary goal in the treatment of <\/span><b>polycythemia vera (PV)<\/b><span style=\"font-weight: 400;\"> is to reduce the risk of <\/span><b>thrombotic events<\/b><span style=\"font-weight: 400;\">.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">For this purpose, <\/span><b>antiplatelet therapy<\/b><span style=\"font-weight: 400;\"> is used, such as <\/span><b>acetylsalicylic acid<\/b><span style=\"font-weight: 400;\"> or, in patients with a history of <\/span><b>deep vein thrombosis<\/b><span style=\"font-weight: 400;\">, <\/span><b>anticoagulant therapy<\/b><span style=\"font-weight: 400;\"> such as <\/span><b>warfarin<\/b><span style=\"font-weight: 400;\"> or <\/span><b>direct oral anticoagulants (DOACs)<\/b><span style=\"font-weight: 400;\">.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">To reduce the number of circulating red blood cells, <\/span><b>periodic phlebotomy (bloodletting)<\/b><span style=\"font-weight: 400;\"> can be performed. This involves removing approximately <\/span><b>400 mL of blood<\/b><span style=\"font-weight: 400;\"> and replacing it with an equivalent volume of saline solution, with the aim of maintaining the <\/span><b>hematocrit below 45%<\/b><span style=\"font-weight: 400;\">.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">In patients older than <\/span><b>60 years<\/b><span style=\"font-weight: 400;\"> or with a history of <\/span><b>thrombotic events<\/b><span style=\"font-weight: 400;\">, or in those who do not achieve adequate blood count control with phlebotomy alone (for example due to elevated white blood cells and platelets), <\/span><b>cytoreductive therapy<\/b><span style=\"font-weight: 400;\"> is used to reduce blood cell production.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">The first line treatment is usually <\/span><b>hydroxyurea<\/b><span style=\"font-weight: 400;\">, an oral drug that reduces cellular replication and is administered chronically at doses adjusted individually for each patient.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">If hydroxyurea is ineffective or not tolerated, alternative treatments include <\/span><b>interferon<\/b><span style=\"font-weight: 400;\">, <\/span><b>JAK2 inhibitors<\/b><span style=\"font-weight: 400;\">, or, in older patients, <\/span><b>alkylating agents<\/b><span style=\"font-weight: 400;\"> such as <\/span><b>busulfan<\/b><span style=\"font-weight: 400;\">.<\/span><\/p>\n<p><b>Interferon<\/b><span style=\"font-weight: 400;\"> is an immunomodulatory agent that reduces the production of red blood cells, white blood cells, and platelets by acting on the immune system. Currently, <\/span><b>pegylated (extended release) formulations<\/b><span style=\"font-weight: 400;\"> are used, administered subcutaneously at intervals of two or more weeks depending on response (for example, <\/span><b>ropeginterferon alfa-2b<\/b><span style=\"font-weight: 400;\">).<\/span><\/p>\n<p><span style=\"font-weight: 400;\">In patients who are intolerant or resistant to hydroxyurea, <\/span><b>ruxolitinib<\/b><span style=\"font-weight: 400;\">, a targeted agent that inhibits the <\/span><b>JAK2 signaling pathway<\/b><span style=\"font-weight: 400;\">, may also 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\"><strong>Essential Thrombocythemia (ET)<\/strong><\/h3>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>Essential thrombocythemia (ET)<\/strong> is a myeloproliferative neoplasm characterized by excessive platelet production, which can lead to abnormalities in blood clotting and an increased risk of thrombosis or, more rarely, bleeding complications.<\/p>\n\n\n\t<div id=\"block_6a47d3a756acd\" 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 at approximately <\/span><b>1.5\u20132.4 cases per 100,000 people<\/b><span style=\"font-weight: 400;\">. The disease is more common in <\/span><b>women<\/b><span style=\"font-weight: 400;\">, and the average age at diagnosis is around <\/span><b>60 years<\/b><span style=\"font-weight: 400;\">, although about <\/span><b>20% of patients are diagnosed before the age of 40<\/b><span style=\"font-weight: 400;\">.\u00a0<\/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><b>Essential thrombocythemia (ET)<\/b> <b>may be asymptomatic<\/b><span style=\"font-weight: 400;\"> or associated with clinical manifestations related to altered blood flow or, in some cases, to <\/span><b>ischemic or thrombotic events<\/b><span style=\"font-weight: 400;\"> caused by vascular occlusion due to blood clots (<\/span><b>thrombi<\/b><span style=\"font-weight: 400;\">).<\/span><\/p>\n<p><span style=\"font-weight: 400;\">The most common symptoms include <\/span><b>headache<\/b><span style=\"font-weight: 400;\">, <\/span><b>paresthesia or abnormal sensations in the fingers, hands, and feet<\/b><span style=\"font-weight: 400;\">, <\/span><b>chest pain<\/b><span style=\"font-weight: 400;\">, <\/span><b>visual disturbances<\/b><span style=\"font-weight: 400;\"> (including transient vision loss or seeing spots), <\/span><b>weakness<\/b><span style=\"font-weight: 400;\">, <\/span><b>dizziness<\/b><span style=\"font-weight: 400;\">, and <\/span><b>bleeding<\/b><span style=\"font-weight: 400;\">, usually mild. Bleeding manifestations may include <\/span><b>epistaxis<\/b><span style=\"font-weight: 400;\">, easy bruising, mild gum bleeding, or gastrointestinal bleeding.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">Despite the increased platelet count, <\/span><b>hemorrhagic events may occur<\/b><span style=\"font-weight: 400;\">, often related to platelet interference with <\/span><b>von Willebrand factor<\/b><span style=\"font-weight: 400;\">, an essential protein in the coagulation process.<\/span><\/p>\n<p><b>Splenomegaly<\/b><span style=\"font-weight: 400;\"> (enlargement of the spleen) may be present, although it is not common.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">The <\/span><b>life expectancy<\/b><span style=\"font-weight: 400;\"> of patients with ET is close to normal, and the overall clinical course is generally benign. Approximately <\/span><b>10% of cases<\/b><span style=\"font-weight: 400;\"> may progress to a more advanced disease form such as <\/span><b>myelofibrosis<\/b><span style=\"font-weight: 400;\">, while transformation to <\/span><b>acute leukemia<\/b><span style=\"font-weight: 400;\"> is rare, occurring in <\/span><b>1\u20133% of cases<\/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\">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 <\/span><b>essential thrombocythemia (ET)<\/b><span style=\"font-weight: 400;\"> is based on clinical evaluation and laboratory findings, in particular the detection of <\/span><b>elevated platelet counts<\/b><span style=\"font-weight: 400;\"> in the blood count, together with the exclusion of causes of <\/span><b>reactive thrombocytosis<\/b><span style=\"font-weight: 400;\">, such as <\/span><b>blood loss, trauma, infection, other neoplasms, or splenectomy<\/b><span style=\"font-weight: 400;\">.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">The disease is associated with <\/span><b>acquired genetic mutations<\/b><span style=\"font-weight: 400;\">, most commonly involving <\/span><b>JAK2<\/b><span style=\"font-weight: 400;\">, the <\/span><b>thrombopoietin receptor gene (MPL)<\/b><span style=\"font-weight: 400;\">, or the <\/span><b>CALR gene<\/b><span style=\"font-weight: 400;\">, which regulates platelet production. Identification of a mutation in one of these genes supports the diagnosis of ET, although the absence of detectable mutations does not exclude it.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">To differentiate ET from other <\/span><b>myeloproliferative neoplasms<\/b><span style=\"font-weight: 400;\">, it is also necessary to perform testing for <\/span><b>BCR\/ABL<\/b><span style=\"font-weight: 400;\">\u00a0and to carry out a <\/span><b>bone marrow biopsy (osteomedullary biopsy)<\/b><span style=\"font-weight: 400;\"> with 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><span style=\"font-weight: 400;\">Thrombotic risk in <\/span><b>essential thrombocythemia (ET)<\/b><span style=\"font-weight: 400;\"> is stratified using the <\/span><b>IPSET (International Prognostic Score for Thrombosis in Essential Thrombocythemia)<\/b><span style=\"font-weight: 400;\"> score, which considers four main factors: <\/span><b>age over 60 years<\/b><span style=\"font-weight: 400;\">, <\/span><b>history of thrombotic events<\/b><span style=\"font-weight: 400;\">, presence of <\/span><b>cardiovascular risk factors<\/b><span style=\"font-weight: 400;\"> (such as smoking, hypertension, and diabetes), and the <\/span><b>JAK2 V617F mutation<\/b><span style=\"font-weight: 400;\">.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">Based on these variables, patients are classified into <\/span><b>low-, intermediate-, or high-risk<\/b><span style=\"font-weight: 400;\"> categories for thrombosis. This stratification supports the selection of the most appropriate therapy to prevent complications.<\/span><\/p>\n<p><b>Low-risk patients<\/b><span style=\"font-weight: 400;\"> (no risk factors) generally do not require antiplatelet or anticoagulant therapy, unless individually indicated.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">In patients at <\/span><b>intermediate or high risk<\/b><span style=\"font-weight: 400;\"> (presence of one or more risk factors), <\/span><b>low-dose acetylsalicylic acid<\/b><span style=\"font-weight: 400;\"> is recommended to reduce the risk of thrombotic events, or <\/span><b>anticoagulant therapy<\/b><span style=\"font-weight: 400;\"> in cases with a history of thrombosis. In addition, <\/span><b>high-risk patients<\/b><span style=\"font-weight: 400;\"> require <\/span><b>cytoreductive therapy<\/b><span style=\"font-weight: 400;\"> to control platelet counts.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">Cytoreductive treatment options include <\/span><b>hydroxyurea<\/b><span style=\"font-weight: 400;\">, <\/span><b>anagrelide<\/b><span style=\"font-weight: 400;\">, or, particularly in younger patients, <\/span><b>pegylated interferon<\/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<\/div>\n\n\t<\/div>\n\n\n\n<h3 class=\"wp-block-heading\"><strong>Myelofibrosis (MF)<\/strong><\/h3>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>Myelofibrosis<\/strong> is a chronic myeloproliferative neoplasm in which normal bone marrow is progressively replaced by fibrous tissue.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">When it develops without a prior history of blood disorders, it is referred to as <strong>primary myelofibrosis<\/strong>. When it evolves from <strong>polycythemia vera<\/strong> or <strong>essential thrombocythemia<\/strong>, it is known as <strong>secondary myelofibrosis<\/strong>.<\/p>\n\n\n\t<div id=\"block_6a47d3a756b98\" 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 approximately <\/span><b>0.5\u20131.3 cases per 100,000 people<\/b><span style=\"font-weight: 400;\">, and it is classified as a <\/span><b>rare disease<\/b><span style=\"font-weight: 400;\">. The average age at diagnosis is around <\/span><b>65 years<\/b><span style=\"font-weight: 400;\">.\u00a0<\/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><b>Idiopathic myelofibrosis<\/b><span style=\"font-weight: 400;\"> may <\/span><b>initially <\/b><span style=\"font-weight: 400;\">be <\/span><b>asymptomatic<\/b><span style=\"font-weight: 400;\">. Over time, symptoms related to <\/span><b>splenomegaly<\/b><span style=\"font-weight: 400;\">, reduced <\/span><b>complete blood count (CBC) values<\/b><span style=\"font-weight: 400;\">, and so called <\/span><b>constitutional symptoms<\/b><span style=\"font-weight: 400;\"> may appear, including <\/span><b>weight loss<\/b><span style=\"font-weight: 400;\">, <\/span><b>general malaise<\/b><span style=\"font-weight: 400;\">, <\/span><b>fever<\/b><span style=\"font-weight: 400;\">, and <\/span><b>night sweats<\/b><span style=\"font-weight: 400;\">.<\/span><\/p>\n<p><b>Splenomegaly<\/b><span style=\"font-weight: 400;\"> may cause <\/span><b>abdominal distension<\/b><span style=\"font-weight: 400;\">, <\/span><b>postprandial fullness<\/b><span style=\"font-weight: 400;\">, and <\/span><b>left flank pain<\/b><span style=\"font-weight: 400;\">.<\/span><\/p>\n<p><b>Anemia<\/b><span style=\"font-weight: 400;\"> may lead to <\/span><b>fatigue<\/b><span style=\"font-weight: 400;\">, <\/span><b>weakness<\/b><span style=\"font-weight: 400;\">, and <\/span><b>dyspnea on exertion<\/b><span style=\"font-weight: 400;\">.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">Alterations in <\/span><b>platelet counts<\/b><span style=\"font-weight: 400;\">, which may be either increased or decreased, can result in <\/span><b>thrombotic or hemorrhagic events<\/b><span style=\"font-weight: 400;\">.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">A reduction in <\/span><b>white blood cells<\/b><span style=\"font-weight: 400;\"> increases susceptibility to <\/span><b>infections<\/b><span style=\"font-weight: 400;\">.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">The clinical course of primary myelofibrosis is variable: some patients experience a slow and stable evolution, while others show a more rapid progression.<\/span><\/p>\n<p><b>Risk stratification systems<\/b><span style=\"font-weight: 400;\">, based on clinical findings, laboratory data, and genetic information, are used to define prognostic groups and guide the most appropriate therapeutic approach for each patient.<\/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><b>Idiopathic myelofibrosis<\/b><span style=\"font-weight: 400;\"> should be suspected in patients presenting with <\/span><b>splenomegaly<\/b><span style=\"font-weight: 400;\"> and abnormalities in the blood count, which may include either increases or decreases in <\/span><b>white blood cells, red blood cells, and platelets<\/b><span style=\"font-weight: 400;\">.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">When the disease is suspected, in addition to the <\/span><b>complete blood count (CBC)<\/b><span style=\"font-weight: 400;\">, the following investigations are indicated:<\/span><\/p>\n<ul>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><b>Peripheral blood smear<\/b><span style=\"font-weight: 400;\">, to evaluate red blood cell morphology and quantify immature circulating cells. Characteristic findings may include <\/span><b>abnormally shaped red blood cells (e.g., dacryocytes)<\/b><span style=\"font-weight: 400;\"> as well as circulating precursors of white blood cells (<\/span><b>myeloblasts<\/b><span style=\"font-weight: 400;\">) and red blood cells (<\/span><b>erythroblasts<\/b><span style=\"font-weight: 400;\">).<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><b>Lactate dehydrogenase (LDH) levels<\/b><span style=\"font-weight: 400;\">, which are often elevated.<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><b>Bone marrow aspiration and biopsy (osteomedullary biopsy)<\/b><span style=\"font-weight: 400;\">, essential for histological evaluation and for assessing the degree of <\/span><b>fibrosis<\/b><span style=\"font-weight: 400;\">.<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><b>Molecular testing for JAK2, CALR, and MPL mutations<\/b><span style=\"font-weight: 400;\">.<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><b>Extended molecular profiling using next generation sequencing (NGS)<\/b><span style=\"font-weight: 400;\"> on peripheral or bone marrow blood to identify additional mutations associated with increased risk of <\/span><b>leukemic transformation<\/b><span style=\"font-weight: 400;\">. This analysis is particularly relevant for prognostic assessment, especially in younger patients who may be candidates for <\/span><b>hematopoietic stem cell transplantation<\/b><span style=\"font-weight: 400;\">.<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><b>Abdominal ultrasound or, if necessary, abdominal CT scan<\/b><span style=\"font-weight: 400;\">, to evaluate liver and spleen size and to exclude <\/span><b>thrombotic involvement of abdominal vessels<\/b><span style=\"font-weight: 400;\">.<\/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. INOC &#8211; Istituto Nazionale Oncologico Candiolo is equipped with a Transplant <a href=\"https:\/\/inoc.it\/en\/medical-area\/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 class=\"wp-block-paragraph\">At INOC &#8211; Istituto Nazionale Oncologico Candiolo, continuous support is provided <strong>before, during, and after treatment<\/strong>, ensuring that each patient is accompanied throughout the entire care and recovery pathway.<\/p>\n\n\n\t<div id=\"block_6a47d3a756c85\" 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><span style=\"font-weight: 400;\">Treatment for <\/span><b>rare sarcomas and tumors<\/b><span style=\"font-weight: 400;\"> may be associated with side effects that can variably impact quality of life. However, these effects can often be mitigated, and in some cases prevented, through specific treatments and appropriate lifestyle measures.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">At INOC &#8211; Istituto Nazionale Oncologico Candiolo, physicians and nurses within the <\/span><b>multidisciplinary team<\/b><span style=\"font-weight: 400;\"> are available to provide patients with comprehensive support in managing the side effects that may occur during the course of treatment.<\/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\">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;\">The cancer patient is often a <\/span><b>vulnerable individual <\/b><span style=\"font-weight: 400;\">who requires continuous support throughout the disease course. When new symptoms arise, whether related to the underlying disease or to treatment-related adverse effects, it is essential that timely specialist evaluation is ensured through a dedicated <\/span><b>\u201cfast track\u201d<\/b><span style=\"font-weight: 400;\"> pathway.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">INOC &#8211; Istituto Nazionale Oncologico Candiolo provides a dedicated support service, available <strong>Monday to Friday from 8:00 a.m. to 5:00 p.m.<\/strong> Patients may contact the <strong>Oncology Day Hospital Secretariat at +39 011.993.3775<\/strong> to report the need for an urgent clinical assessment. The referring specialist is then promptly informed and will contact the patient to ensure timely evaluation and appropriate management.<\/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 class=\"isSelectedEnd\">Cancer patients often face complex needs that extend beyond the treatment of the disease itself, requiring comprehensive, multidisciplinary support to address both medical and quality-of-life challenges.<\/p>\n<p>At INOC \u2013 Istituto Nazionale Oncologico Candiolo, patients who need additional support have access to a team of specialists across multiple disciplines. This integrated approach includes<strong> nutritional counseling, physical rehabilitation, pain management, and the treatment of other cancer-related conditions,<\/strong> ensuring <strong>personalized<\/strong> and <strong>holistic care<\/strong> throughout the patient journey.<\/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 INOC &#8211; Istituto Nazionale Oncologico Candiolo, conducts <\/span><b>information and orientation interviews for 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 <strong>Wednesdays and Fridays from 9 a.m. to 1 p.m. \u2013 Phone: +39 011.993.30<\/strong><\/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 class=\"isSelectedEnd\">In many cases, <strong>chronic myeloproliferative neoplasms can be safely monitored over time without the need for immediate active treatment<\/strong>.<\/p>\n<p class=\"isSelectedEnd\">Throughout the follow-up period, patients undergo <strong>regular clinical assessments and diagnostic tests<\/strong> to monitor <strong>disease progression<\/strong>, evaluate the <strong>effectiveness of therapies<\/strong>, identify <strong>potential side effects<\/strong>, and assess <strong>overall health and functional recovery<\/strong>.<\/p>\n<p class=\"isSelectedEnd\"><strong>Follow-up visits play a crucial role in the early detection of disease changes<\/strong>, enabling <strong>timely and appropriate intervention<\/strong> when necessary. They also provide patients with an important opportunity to <strong>discuss concerns, ask questions, and maintain an ongoing dialogue with their specialist team<\/strong>.<\/p>\n<p>The follow-up plan is <strong>personalized for each patient<\/strong> and varies according to the <strong>type of chronic myeloproliferative neoplasm<\/strong>, the <strong>treatment received<\/strong>, the <strong>response achieved<\/strong>, and the patient&#8217;s <strong>individual clinical characteristics and needs<\/strong>.<\/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=\"multi-disciplinary-team\" class=\"h2_anchor\"><\/span><strong>Multi Disciplinary Team<\/strong><\/h2>\n\n\n\n<p class=\"wp-block-paragraph\">Every cancer requires a <strong>multidisciplinary approach<\/strong> at all stages of disease management. At INOC &#8211; Istituto Nazionale Oncologico Candiolo, this is provided by a team of specialists from various clinical and surgical departments, known as the <strong>GIC <\/strong>(Interdisciplinary Care Group or MDT). The GIC ensures that each patient is supported throughout the diagnostic and therapeutic process, including arranging and coordinating examinations and maintaining communication with the patient and their family.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">For each patient, the GIC (MDT) defines and shares a personalized care pathway based not only on the type and stage of the tumor but also on the patient\u2019s individual characteristics. The goal is to achieve the best possible outcomes both oncologically and functionally, while maintaining a high quality of life.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">The Group<strong> also collaborates closely with the Institute\u2019s researchers<\/strong> to provide patients with rapid access to the latest 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 class=\"wp-block-paragraph\">The diagnostic-therapeutic pathway of chronic myeloproliferative syndromes at <strong>INOC &#8211; Istituto Nazionale Oncologico Candiolo<\/strong> involves several clinical divisions, including:<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><a href=\"https:\/\/inoc.it\/en\/medical-area\/medical-oncology\/\" type=\"medical-area\" id=\"5169\">Medical Oncology<\/a><\/li>\n\n\n\n<li><a href=\"https:\/\/inoc.it\/en\/medical-area\/oncology-day-hospital\/\" type=\"medical-area\" id=\"5183\">Day Hospital<\/a><\/li>\n\n\n\n<li><a href=\"https:\/\/inoc.it\/en\/medical-area\/laboratory-analysis\/\" type=\"medical-area\" id=\"5113\">Laboratory Analysis<\/a><\/li>\n\n\n\n<li><a href=\"https:\/\/inoc.it\/en\/medical-area\/pathologic-anatomy\/\" type=\"medical-area\" id=\"5118\">Anatomical Pathology<\/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 class=\"wp-block-paragraph\">Clinical research in chronic myeloproliferative neoplasms is focused on <strong>optimizing available therapies<\/strong> through the strategic use of <strong>different generations of drugs and molecular inhibitors<\/strong>, often in combination, with the aim of <strong>improving outcomes and preventing treatment resistance<\/strong> in both <strong>Chronic Myeloid Leukemia (CML)<\/strong> and other myeloproliferative malignancies.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">For patients with <strong>Chronic Myeloid Leukemia<\/strong> who achieve a <strong>deep molecular response<\/strong>, numerous clinical studies have demonstrated the possibility of <strong>safely discontinuing therapy in a significant proportion of cases<\/strong>. This represents a major advancement, transforming what was once considered a lifelong treatment into a <strong>time-defined therapeutic approach<\/strong> for selected patients.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">In <strong>polycythemia vera<\/strong>, innovative therapies with <strong>novel mechanisms of action<\/strong> are currently under investigation, including agents that <strong>control hematocrit levels by modulating iron metabolism<\/strong>.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">In <strong>essential thrombocythemia<\/strong>, research is evaluating <strong>new interferon formulations<\/strong> as well as <strong>monoclonal antibodies targeting calreticulin<\/strong>, with the goal of expanding therapeutic options and improving disease control.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">In <strong>myelofibrosis<\/strong>, several promising therapies are being studied to target the different biological pathways involved in disease development and progression. These include <strong>next-generation tyrosine kinase inhibitors<\/strong>, such as <strong>pacritinib<\/strong>, as well as <strong>BET inhibitors (pelabresib)<\/strong> and <strong>BCL-2 inhibitors (navitoclax)<\/strong>. By targeting proteins involved in <strong>DNA transcription, cellular proliferation, and cancer cell survival<\/strong>, these therapies may help eliminate malignant cells and improve patient outcomes.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Additional investigational agents include <strong>MDM2 inhibitors<\/strong>, such as <strong>navtemadlin<\/strong>, which promote <strong>programmed cancer cell death (apoptosis)<\/strong> and may be effectively combined with <strong>JAK inhibitors<\/strong> through complementary mechanisms of action. <strong>BTK inhibitors<\/strong> are also being explored for their ability to <strong>modulate inflammatory pathways<\/strong>, potentially benefiting patients with <strong>significant inflammatory symptoms and splenomegaly (enlarged spleen)<\/strong>.<\/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 class=\"wp-block-paragraph\">At INOC \u2013 Istituto Nazionale Oncologico Candiolo, every patient with bladder cancer is treated in a highly specialized manner, thanks to the synergistic work of the dedicated\u00a0<a href=\"https:\/\/inoc.it\/en\/pathology\/chronic-myeloproliferative-neoplasms\/#multi-disciplinary-team\">Interdisciplinary Care Group (GIC).<\/a><\/p>\n\n\n\t<div id=\"block_6a47d3a756d35\" 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><span style=\"font-weight: 400;\">Thanks to the large number of cases treated each year, INOC \u2013 Istituto Nazionale Oncologico Candiolo is a <\/span><b>national reference<\/b><span style=\"font-weight: 400;\"> center for the care of this disease. This extensive experience allows us to manage even the most complex cases, always using a <\/span><b>personalized approach<\/b><span style=\"font-weight: 400;\">, tailored to the clinical and individual profile of each patient.<\/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\">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><span style=\"font-weight: 400;\">Establishing a treatment plan always begins with an accurate and timely diagnosis. Patients have access to <\/span><b>state-of-the-art imaging technologies<\/b><span style=\"font-weight: 400;\">, such as ultrasound, contrast-enhanced CT, MRI, and cholangio-RM, which are critical for accurately assessing the extent of the tumor.<\/span><\/p>\n<p><b>Advanced laboratory tests<\/b><span style=\"font-weight: 400;\">, including molecular analyses, are also available to help define biological features of the disease and guide treatment choices.<\/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\">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><span style=\"font-weight: 400;\">When appropriate, surgeries are performed using <\/span><b>minimally invasive<\/b><span style=\"font-weight: 400;\">, laparoscopic or robotic <\/span><b>techniques<\/b><span style=\"font-weight: 400;\">. These approaches involve the use of camera-equipped instruments introduced into the abdomen through small incisions, thus reducing surgical trauma. <\/span><b>Benefits<\/b><span style=\"font-weight: 400;\"> to the patient include <\/span><b>shorter hospital stay times, faster recovery, and lower risk of complications compared with traditional open surgery.<\/b><\/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<p><span style=\"font-weight: 400;\">As an IRCCS (Scientific Institute for Research, Hospitalization, and Healthcare), INOC \u2013 Istituto Nazionale Oncologico Candiolo combines clinical care with a strong focus on scientific research. Patients can be considered for <\/span><a href=\"https:\/\/inoc.it\/en\/pathology\/chronic-myeloproliferative-neoplasms\/#clinical-studies\"><b>participation in active clinical trials<\/b><\/a><span style=\"font-weight: 400;\">, offering access to innovative therapies not yet available in standard practice. This integration of care and research is a distinctive strength that translates into tangible benefits for patients.<\/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\">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><a href=\"https:\/\/inoc.it\/en\/pathology\/chronic-myeloproliferative-neoplasms\/#multi-disciplinary-team\"><span style=\"font-weight: 400;\">The Interdisciplinary Care Group<\/span><\/a><span style=\"font-weight: 400;\"> (GIC or MDT) <\/span><b>supports the patient at every stage<\/b><span style=\"font-weight: 400;\">: from diagnosis, through treatment, to follow-up. Special attention is paid to<\/span><b> nutritional support, psychological health and reintegration into daily life.<\/b><span style=\"font-weight: 400;\"> The organization of checkups, examinations, and treatment is designed to ensure <\/span><b>continuity, serenity, and a humane, caring<\/b><span style=\"font-weight: 400;\"> approach to each patient\u2019s needs.<\/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","protected":false},"featured_media":0,"template":"","meta":{"_acf_changed":false,"_relevanssi_hide_post":"","_relevanssi_hide_content":"","_relevanssi_pin_for_all":"","_relevanssi_pin_keywords":"","_relevanssi_unpin_keywords":"","_relevanssi_related_keywords":"","_relevanssi_related_include_ids":"","_relevanssi_related_exclude_ids":"","_relevanssi_related_no_append":"","_relevanssi_related_not_related":"","_relevanssi_related_posts":"","_relevanssi_noindex_reason":"","footnotes":""},"class_list":["post-5230","pathology","type-pathology","status-publish","hentry"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v28.1 - 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