# Care pathway for myeloproliferative diseases On this page you will find information about the different stages of examinations and treatment of myeloproliferative diseases at HUS. ## [ From symptoms to diagnosis ](#from-symptoms-to-diagnosis) ## [ Getting a diagnosis ](#getting-a-diagnosis) ## [ Treatments ](#treatments) ## [ After treatments ](#after-treatments) ## [ Support and information ](#support-and-information) ## From symptoms to diagnosis Primary health care ### A symptom appears or an abnormal finding is made --- Myeloproliferative diseases are malignant blood diseases characterized by excessive production of blood cells. The disease group includes polycythemia vera (PV), essential thrombocytosis (ET), and myelofibrosis (MF). The disease is usually discovered by chance on the basis of blood tests taken for other reasons. Sometimes the disease is investigated for symptoms such as general tiredness, headache, a heavy feeling in the head, or exceptionally profuse night sweats. A typical symptom is itching of the skin, which worsens especially after showering or going to the sauna. Myeloproliferative diseases also increase the risk of blood vessel occlusions. The disease can be discovered when investigating the cause of venous or arterial thrombosis. Myelofibrosis (MF) is characterized by an enlarged spleen. This may be accompanied by pain in the flank or upper abdominal area on the left and a feeling of rapid filling of the stomach when eating. ### Required additional examinations in primary health care and a physician’s assessment --- The initial examinations may be performed at a health center, in occupational health care, or at a private clinic. ### A physician writes a referral --- If an abnormal examination result or finding is made, the physician will make a referral to HUS. ## Getting a diagnosis Comprehensive Cancer Center and other specialized medical care ### The referral arrives at specialized medical care and an invitation is sent to the patient --- When your referral arrives at HUS, we will direct it to an internal medicine outpatient clinic at the Jorvi, Peijas, Helsinki Laakso, Malmi, or Lohja Hospital, or to a hematology outpatient clinic at the Comprehensive Cancer Center, Hyvinkää Hospital, or Porvoo Hospital, depending on your place of residence. Our specialist will plan the necessary additional examinations based on your referral. You will be informed of the time of the examinations and appointments by letter. You can also find this information in the Maisa portal, which enables electronic transactions with your treatment unit. ### If necessary, additional examinations can be carried out --- To confirm a myeloproliferative disease, we will take a bone marrow sample from you. We take a bone marrow sample from the rear of the pelvic bone under local anesthesia. The sampling takes about 20–30 minutes. You will receive more detailed instructions on the procedure in your appointment letter. In addition to the bone marrow samples, we also take blood tests. ### Appointment with a physician and a nurse --- Results for some of the bone marrow sample tests are completed quickly, but some take 3–4 weeks to complete. For this reason, we will book you a physician’s appointment approximately one month after the bone marrow sample is taken, so that all the examination results would be available at the appointment. This allows us to confirm the diagnosis at the appointment and draw up a treatment plan with you. You may bring a loved one to the appointment if you wish. At the physician’s appointment, we will ask you about your other illnesses and symptoms related to your disease. Your physician will examine you by listening to your heart and breathing sounds and palpating your stomach. After the examinations, you will discuss the diagnosis and treatment plan with your physician. After the physician’s appointment, you will meet the nurse at the outpatient clinic either on the same day or within a few weeks. The nurse will go through matters related to your blood disorder with you and provide more detailed guidance, for example, on the medication you will be starting to take. ## Treatments Specialized medical care ### Treatment begins --- Treatments for different myeloproliferative diseases differ slightly from each other. With the help of the treatments, we aim to alleviate possible symptoms and reduce the risk of complications, such as blood clots. To reduce the risk of thrombosis, we often include acetylsalicylic acid tablets in the treatment and, if necessary, use medication to normalize other risk factors, such as blood pressure, cholesterol and glucose levels. If necessary, we support patients that smoke in quitting smoking. Asymptomatic essential thrombocytosis may be monitored without medical treatment for up to several years, but we will start treatment of polycythemia vera as soon as the diagnosis is confirmed. If necessary, we will start medication to lower elevated blood counts. You can read more about different diseases and their treatment in the Cancer Hub in Health Village (Terveyskylä online service; content currently not available in English): - [Essential thrombocytosis ](https://www.terveyskyla.fi/syopatalo/veritaudit/essentielli-trombosytoosi) - [Myelofibrosis](https://www.terveyskyla.fi/syopatalo/veritaudit/myelofibroosi) - [Polycythemia vera](https://www.terveyskyla.fi/syopatalo/veritaudit/polysytemia-vera) ### Healing and follow-up --- There is no curative drug treatment for myeloproliferative diseases. With the available treatments, the life expectancy of patients with essential thrombocytosis and polycythemia vera has improved significantly and does not differ much from the life expectancy of healthy people. Unlike other myeloproliferative diseases, the course of primary myelofibrosis can be rapid and the prognosis poor. For this reason, the possibility of stem cell transplantation from a healthy donor is being investigated for high-risk myelofibrosis patients in good condition. It is the only curative treatment method. We monitor the treatment response in all forms of the disease with blood samples taken every few months. We carry out follow-up both remotely by phone and at appointment visits. ### If the disease recurs or treatments do not help --- Myeloproliferative diseases are chronic in nature. However, they can change their form over the years, in which case we change or intensify your treatment. As a rule, your treatment is always continuous. Of the diseases, essential thrombocytosis and polycythemia vera can progress to myelofibrosis. This is referred to as secondary myelofibrosis, which we treat in the same way as primary myelofibrosis. In rare cases, all of the above diseases can progress to acute leukemia. In this case, we will follow the principles of treatment for acute leukemia. If a treatment that slows down the disease cannot be used due to your general condition or the resistance of the disease, we will proceed with symptomatic treatment. [At the Clinical Trial Unit](/en/patient/hospitals-and-other-units/comprehensive-cancer-center/clinical-pharmaceutical-research-unit "Clinical Trial Unit, Comprehensive Cancer Center") we offer an increasing number of patients the opportunity to be among the first to receive new research treatments. ### Palliative care and psychosocial support --- Good palliative care and psychosocial support are an integral part of cancer treatment. The Palliative Care Center provides help in the stressful life situation caused by the illness or cancer treatments. We provide help in treating difficult symptoms and offer conversational support. A decision on palliative, i.e. symptomatic, treatment is made when the focus of your treatment shifts from slowing down the progression of cancer to managing the symptoms. In this case, treatment aims to alleviate symptoms and, as far as possible, to ensure a good and active life with the illness until death. Typically, at this stage, your care responsibility is transferred to the palliative care unit or to the palliative outpatient clinic or hospital-at-home unit in your area. [ Read more about the Palliative Care Center ](/en/patient/hospitals-and-other-units/comprehensive-cancer-center/palliative-center-comprehensive) ## After treatments ### Follow-up in specialized medical care ends --- Your illness will be treated at HUS, and follow-up will not be terminated unless the treatment is changed to palliative care. An exception to this rule are young patients with essential thrombocytosis (ET), whose follow-up we often transfer to primary health care until it is time to start their treatment. You may need to start treatment when your platelet counts increase significantly, you are diagnosed with a blood vessel occlusion, or you are over 61 years of age. ## Support and information Getting cancer is a big life change. Here you will find information and support for living with the disease. ### [ Support during cancer treatment ](/en/patient/treatments-and-examinations/support-during-cancer-treatment) Here you can find discussion help and support for rehabilitation for cancer patients.  ### [ Instructions and additional information for cancer patients ](/en/patient/treatments-and-examinations/cancer/instructions-and-additional-information-cancer-patients) Here you will find our patient guide and more information about cancer treatments.  Updated: 10.10.2024