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The American Society of Hematology (ASH) has released guidelines on the management of myelofibrosis, a rare and life-threatening blood cancer. The new guidelines recommend expanded molecular testing, early transplant referral, appropriate use of JAK inhibitors and aspirin therapy in select patients.

Myelofibrosis is a type of myeloproliferative neoplasm, a group of chronic blood cancers in which the bone marrow overproduces different types of blood cells. In myelofibrosis, the bone marrow—or spongy inside of the bone that produces blood cells—develops scar tissue, impacting its ability to make enough healthy blood cells. Myelofibrosis affects only a small number of people, with estimates that up to six per 100,000 people are living with the disease.

Individuals with myelofibrosis are at risk of developing acute myeloid leukemia and other serious, disease-related complications, including enlargement of the spleen (an organ that helps fight infection and filter blood) and development of blood clots.

Myelofibrosis can often be managed over time with medications, including JAK inhibitors, which can help reduce spleen enlargement and ease other symptoms of the disease. Currently, the only potentially curative treatment is allogeneic stem cell transplant, in which a patient's faulty cells are replaced with healthy, blood-forming stem cells from a donor.

ASH's evidence-based guidelines help address decision-making gaps in the care of myelofibrosis. Because myelofibrosis is rare and progresses, approaches to treatment can vary substantially among clinicians.

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