Myelofibrosis

Myelofibrosis - What it is

Myelofibrosis is a type of myeloproliferative neoplasms which is rare chronic blood cancers because of increased fibrosis or scarring in the bone marrow resulting in subnormal blood counts and an enlarged spleen. Myelofibrosis can happen on its own (primary myelofibrosis) or as a sequalae from another myeloproliferative neoplasm like polycythemia vera or essential thrombocythemia (secondary myelofibrosis.

Myelofibrosis - Symptoms

Myelofibrosis may represent with no symptoms, but some may develop symptoms such as:

Fatigue and tiredness because of anamia
Enlarged spleen, leading to symptoms such as abdominal pain and getting full easily after a meal.
Easy bruising or bleeding
Loss of appetite and weight
Bony pains

For those without symptoms, abnormalities may be detected after a routine blood test.

Myelofibrosis - How to prevent?

Many patients with myelofibrosis are recognized incidentally during routine blood test or blood counts testing for other disorders.

Currently there are also no proven preventive strategies for essential thrombocythemia.

However, adopting a healthy lifestyle may help to prevent disease. This includes:

Eating a diet rich in fruits and vegetables
Quitting or not starting smoking
Limiting alcohol consumption
Regular exercise

Myelofibrosis - Causes and Risk Factors

Myelofibrosis occurs when there is a mutation in genes which are involved in the regulation of blood cell production. Specific mutations include JAK2, Calreticulin and MPN gene mutations. The cause of the gene mutation is unknown and is usually not an inheritable mutation.

Myelofibrosis - Diagnosis

To make a diagnosis of myelofibrosis, a bone marrow aspiration and trephine biopsy is done to evaluate the degree of fibrosis. Sample from the blood or bone marrow samples may be used to evaluate for gene mutations. This helps to confirm the diagnosis and also give more information regarding the prognosis and guide treatment options.

Myelofibrosis - Treatments

Issues in patients with myelofibrosis include:

Anaemia
Treatment for anaemia includes danazol, erythropoietin, immunomodulatory agents (iMiDs) and blood transfusion
High blood counts: hydroxyurea, anagrelide, PEGylated interferon
Enlarged spleen: JAK inhibitors (ruxolitinib), radiotherapy to the spleen, splenectomy
Constitutional symptoms such as fatigue and night sweats: ruxolitinib

For younger patients with advanced or poor risk myelofibrosis, allogeneic hematopoietic stem cell transplant may be a consideration because transplant is the only treatment that can potentially cure myelofibrosis.

SGH has extensive expertise in treating MPNs. As the largest hospital in Singapore treating MPNs, it is a one stop comprehensive care centre with clinical and laboratory facilities for diagnosis and monitoring of treatment. We offer state of the art therapies with the possibility of participation in local and international clinical trials. Patients receive holistic care, individualized to their condition and preferences.