Secondary Myelofibrosis

Secondary myelofibrosis is a type of myelofibrosis (MF) and myeloproliferative neoplasm (MPN) that develops in people who have other MPNs such as polycythemia vera (PV) or essential thrombocythemia (ET) first. MF that develops on its own in people who have not been diagnosed with other MPNs is called primary myelofibrosis (PMF). MF is a rare type of blood cancer that causes excessive scar tissue to form in the bone marrow, disrupting the body’s normal production of blood cells. The blood cells that are produced as a result of the fibrosis are abnormal.

There are two different types of secondary myelofibrosis which is determined by the type of MPN that occurred before MF did. These two types are post-PV myelofibrosis and post-ET myelofibrosis. Post-PV myelofibrosis occurs with polycythemia vera, while post-ET myelofibrosis occurs with essential thrombocythemia. According to a paper in the American Journal of Managed Care (AJMC), 15 percent of patients with PV may develop myelofibrosis, and only a small number of patients with ET will develop myelofibrosis.

Although primary and secondary myelofibrosis occur in different ways, the symptoms, complications, and treatments can be similar.

Secondary Myelofibrosis Symptoms

Secondary myelofibrosis symptoms are often caused by an enlarged spleen or as a result of an insufficient numbers of normal blood cells, such as platelets, red blood cells, and white blood cells. Common symptoms include:

• Tiredness, weakness
• Easy bleeding or bruising
• Fullness, or pain in the upper left abdomen
• Night sweats
• Weight loss
• Itching
• Susceptibility to infection
• Joint and/or bon pain
• Fever

Secondary Myelofibrosis Treatment

The treatment options for secondary myelofibrosis vary from patient to patient depending on the severity of the cancer case and symptoms a patient is experiencing. It is not uncommon for patients who do not experience symptoms to wait to receive treatment until symptoms occur. It’s important to consult with a doctor and receive the right testing to determine which treatment option is best.

Available treatments for secondary myelofibrosis include:

• Jakafi (ruxolitinib) – the first approved drug by the FDA for MF patients, which inhibits the activity of the proteins JAK2 and JAK1, which are mutated in 50-60 percent of myelofibrosis patients.
• Inebric (fedratinib) – a treatment for adult patients with intermediate to high-risk secondary myelofibrosis, which is determined by a patient’s doctors during diagnosis.
• Allogenic Stem Cell Transplantation (ASCT) – the only curative treatment for MF, which requires donor stem cells to be transferred to the patient. Not all patients are eligible for ASCT.
• Clinical trials – JAK inhibitors, epigenetic drugs, pomalidomide, and everolimus are novel treatments being studied in clinical trials as possible future FDA-approved treatments for myelofibrosis.

Consult with your doctor to determine which treatment options are available to you.

Resources:

Myeloproliferative Disorders and Myelofibrosis | AJMC

Primary Myelofibrosis Overview | MPNRF (mpnresearchfoundation.org)

Myelofibrosis; Causes, Symptoms, Treatment & Prevention (clevelandclinic.org)