Myeloproliferative Neoplasms (previously known as myeloproliferative disorders) are a rare group of blood disorders in which there is overproduction of one or more of the cell types in the bone marrow (red blood cells, white blood cells or platelets). The symptoms of MPN are due to the raised blood counts as well as the inflammation caused by the rising white cells. Leukaemia refers to a specific condition where the production of white blood cells in the bone marrow is abnormal, resulting in an overproduction of white cells. Acute leukaemia is a separate and serious life-threatening condition, but some forms of chronic leukaemia are considered a form of Myeloproliferative Neoplasm.

A person’s recovery or response to therapy depends on their type of MPN, age, and general health. The major risks of MPNs are related to 1) risk of blood clots (heart attacks or strokes) and 2) risk of transformation to acute leukaemia.

For patients will less aggressive forms of MPN, such as essential thrombocytosis (raised platelet counts) or polycythaemia ruba vera (raised haemoglobin), the relative risk of transformation to leukaemia is low, and the risk of blood clots can be managed and as such the long term prognosis of these groups of patients tends to be good.

In contrast, patients with myelofibrosis, an MPN with increased scarring of the bone marrow, can present with low blood counts, enlarging spleen, and a higher risk of progression to acute leukaemia. The survival of these patients can often be 1-2 years without a bone marrow transplantation.

Myeloproliferative Neoplasms (MPNs) are a group of rare, chronic blood cancers caused by the excess production of different blood cells by the bone marrow. MPNs are not a form of autoimmune disorder, but it is recognised that patients with MPNs may be more predisposed to autoimmune conditions such as inflammatory bowel disease, lupus, aplastic anaemia, psoriasis, and autoimmune haemolytic anaemia.

No, Myeloproliferative Disorders are not hereditary.

Polycythaemia Vera (PV) is the most common Myeloproliferative Disorder. However, it still is a relatively rare condition. It is characterised by the excessive production of normal red blood cells. In 95% of patients with PV, the condition is caused by a change in a gene known as JAK2. It commonly affects patients aged 50-60 or above. 

Patients may present with symptoms such as headaches, fatigue, itchy skin or even a reddened appearance of the skin due to the excessive production of red cells.

MPNs are a group of blood conditions that are classified as cancers. MPNs can cause increased production of one or more of the cell types produced in the bone marrow. This is in contrast with leukaemia which is a blood cancer due to abnormalities in the production of white cells in the bone marrow. Some chronic leukaemias are classified as MPNs (such as chronic myeloid leukaemia and chronic myelomonocytic leukaemia). 

More aggressive forms of MPNs can also progress to acute leukaemia over time.

The most common MPNs are:

1. Polycythaemia vera (PV) which is characterised by increased production of red blood cells.
2. Essential thrombocytosis (ET) which is characterised by increased production of platelets
3. Myelofibrosis (Primary or Secondary) which is characterised by increased fibrosis (scarring) of the bone marrow, often with an enlarging liver or spleen over time as a result.

The primary complications of MPNs are :

1. Generalised MPN-related symptoms including fatigue, headaches, itching (pruritus), and bone pain.
2. Increased risk of blood clots (thrombosis) – this includes deep vein thrombosis (commonly in legs, but it can also happen in less common veins in the abdomen), heart attacks or strokes.
3. Increased risk of transformation to acute leukaemia over time.

Myeloproliferative Neoplasms (MPNs) are types of blood cancer that begin with an abnormal mutation (change) in a stem cell in the bone marrow. The change leads to an overproduction of any combination of white cells, red cells and platelets. Many of the MPNs are caused by mutations of genes in the bone marrow, with mutations in the JAK2 gene being the most common driver for MPN.

Early symptoms of myeloproliferative neoplasms may include headaches, blurred vision, fatigue, weakness, dizziness, itchiness (pruritus), night sweats, and raised blood pressure (hypertension).

Antiplatelet drugs, Hydroxyurea, Interferon and Ruxolitinib are some drug therapies used to treat MPN.

A Venesection is a very safe and straightforward procedure, but some patients may experience side-effects including:

• Discomfort or pain at the site of needle insertion
• Slight bleeding after the needle is removed
• Bruising at the site of needle insertion
• Dizziness. Some patients may develop low blood pressure and experience dizziness during and for a short period after the procedure due to the reduced blood volume and the resulting drop in blood pressure. This should wear off with rest. Patients should get up slowly to avoid feeling dizzy.
• Tiredness – Some patients may feel tired or “washed out” for a few days after the procedure. This effect should also wear off with rest.

If patients require frequent blood transfusions over several years, they may develop iron overload which could result in damage to the heart, liver, and other vital organs. Iron chelation therapy that involves the removal of excess iron from the body can help to minimise the risk of such iron toxicity.

Side effects depend primarily on the type of drug that is given and what it targets. 

The commonly reported side effects include:

• Fatigue
• Diarrhoea
• Reduced appetite
• Skin rashes
• Abnormal changes in smell or taste
• Sensory abnormalities
• Blood tests abnormalities

There are a number of complications that can occur after a stem cell transplant. These include both short term and long term complications.

Patients may experience short term complications such as mouth and throat pain, vomiting or nausea, infection, or graft-versus-host-disease (GVHD). Longer-term complications include disease relapse, GVHD, cardiac and hormonal changes, as well as secondary cancers.

Our transplant team will work closely with you through the different phases of your transplant to monitor, prevent and manage any such complications.

Long-term side effects following a bone marrow transplant vary depending on several factors. For example, the type of transplant, pre-transplant chemotherapy, the patient’s age and health, and whether graft-versus-host-disease (GVHD) is present may all affect the potential side effects experienced by a patient.

Some possible long-term side effects include organ damage, reduced fertility, relapse, second cancer, cataracts, and hormonal changes.