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Myeloproliferative tumors:

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They are called chronic myeloproliferative neoplasms and the concept of is inherent in the name something you will have to live with for the rest of your life They are rare diseases that can accompany the patient for years without aggravating, requiring only periodic checkups or therapies, more or less aggressive depending on the case. A diagnosis of one of these blood cancers, which make them sick every year two thousand Italians, it is not necessarily synonymous with giving up daily life. It doesn’t necessarily mean saying goodbye to small or big goals: from work to marriage, from traveling to… ascent of Mont Blancthe challenge it has set itself Francesca Masica patient who will attempt to climb to the top of more than 4,000 meters in July (2 years after the transplant for the treatment of the myelofibrosis from which he suffers).

Up to the top. Francesca’s challenge over 4,000 meters

Tuscany, 46, psychologist by profession and passionate about trekking, Francesca discovered the pathology in 2016 and after keeping her under pharmacological control for more than 4 years, she had to undergo a transplant in 2020. “I am a mountain lover and since the age of 25 I spend my holidays hiking – says Masi, who tells about his venture in the Facebook column « Up “-. One of my greatest wishes was to be able to climb Mont Blanc Now I am well and in July I will try to reach the top, seal this moment and celebrate the desire to live. It will be a real climb after the metaphorical one, because so many sick people, like me, they climb mountains of difficulty to overcome diseaseFrancesca’s challenge and her return to life are the focus of the 2022 edition of “MIELO-Spieghi”, the information campaign on chronic myeloproliferative neoplasms promoted by Novartis in collaboration with the Italian Association of Patients with Myeloproliferative Diseases ( AIPAMM), with the patronage of AIL (Italian Association of Leukemia, Lymphomas and Myeloma) and MPN Advocates Network. “Realizing that you are not overwhelmed by your illness, your symptoms, brings a sense of pride and pride that, if the tragic situation was not there, would be an indescribable source of self-esteem and joie de vivre,” adds Masi. a patient should be concerned about psychologically coping with his illness

What are Myeloproliferative Neoplasms?

“Myeloproliferative neoplasms are rare tumors that affect the bone marrow – he explains” Francesco Passamontic, professor of hematology at the University of Insubria in Varese -, may have a slow but progressive trend. Symptoms can also be important, but some therapeutic innovations, such as JAK2 inhibitors, have improved the course of these diseases: now patients can build a new daily life and set small, big life goals for themselves“In short, if there is still no cure, these are cancers that can be controlled for years.” Polycythemia vera is characterized by the progressive increase of red blood cells – he explains Alessandro Maria Vannucchi, professor of hematology at the University of Florence and director of hematology at Careggi -; essential thrombocythemia involves an excessive increase in platelet count and involves myelofibrosis change in blood cell count, combined with an increase in the volume of the spleen, weight loss, night sweats, deep fatigue, so much so that normal work and daily activities are limited. Today, therapeutic innovations have changed the history of chronic myeloproliferative diseases and it is now possible to actively live with them, as the challenge of Francesca Masi attests ».

the therapies

Since they are rare pathologies, they are little known: It can therefore be difficult for patients to arrive at a correct diagnosis and a referral center with expert hematologists who prescribe the most appropriate therapy. However, a correct therapeutic approach and the doctor-patient alliance play a fundamental role in involvement of the patient in their own care process (which lasts a lifetime). MPN Tracker, a new and intuitive online tool that enables symptom control so you have a clear overall picture of the disease to share with your reference hematologist. Current treatments are based on: bloodletting for those who suffer from polycythemia, onaspirin to prevent thrombosis and further chemotherapy drugs to control the abnormal increase in the number of blood cells, to reduce the risk of thrombosis or to prevent the growth of the spleen. «The only solution to cure is stem cell transplant from a healthy donor, reserved for those who have myelofibrosis with a poor prognosis and are younger than 60 – Vannucchi adds. And then there are the new drugs, targeting those genetic changes that we know are responsible for the disease. Today, they enable patients to survive and reduce debilitating symptoms, enabling in most cases the return to daily activities and work and a normal social life ».

Source: Corriere

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