Care pathway for myeloproliferative diseases

Myeloproliferative diseases are malignant blood diseases characterized by excessive production of blood cells. This group of diseases includes:

• Polycythemia vera (PV)
• essential thrombocythemia (ET)
• 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.

The initial examinations may be performed at a health center, in occupational health care, or at a private medical clinic.

If an abnormal examination result or finding is made, the physician will make a referral to HUS.

When your referral arrives at HUS, we will refer you based on your place of residence

• to the internal medicine outpatient clinic at Jorvi, Peijas, Raseborg, Malmi, or Lohja Hospital
• or to the hematology outpatient clinic at the Comprehensive Cancer Center, Hyvinkää Hospital, or Porvoo Hospital.

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 service, which enables electronic transactions with your treatment unit. 

To confirm a diagnosis of myeloproliferative disease, we will take a bone marrow sample from the back of your 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.

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. This allows us to confirm the diagnosis at the appointment and draw up a treatment plan. You may bring a family member 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 by palpating your stomach. After the examinations, you will have a discussions about 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 you will receive m

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 thrombocythemia 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 thrombocythemia
• Myelofibrosis
• Polycythemia vera

A multiprofessional treatment team is in charge of the treatment of myeloproliferative disorders. You can reach your treatment team in the Maisa Portal and by phone. You will find the contact information for the unit treating you on the invitation letter.

• The Comprehensive Cancer Center helpline at 09 471 74900 is open Monday through Friday from 7:30 a.m. to 4:00 p.m.
• If your condition deteriorates suddenly or your fever rises above 38 degrees Celsius, contact the 24-hour emergency Medical Helpline at 116 117 (toll-free) or the nearest hospital with on-call services.
• In an emergency, always call 112.

There is no curative drug treatment for myeloproliferative diseases. With the available treatments, the life expectancy of patients with essential thrombocythemia and polycythemia vera has improved significantly and does not differ much from the life expectancy of healthy patients. 

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. Follow-up can be carried out both remotely by phone and during appointment visits.

During rehabilitation, it is important to be aware of your fatigue and to pace yourself according to your energy levels. By exercising regularly, you can reduce the risk of blood clots. As part of the counseling, you will also receive support in adopting lifestyle habits that promote cardiovascular health.

We will treat your condition at HUS and will not discontinue your follow-up care unless treatment is switched to a symptomatic treatment plan.

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.