Blood and bone marrow cancers take many forms, each affecting how the body produces and regulates blood cells. While leukemia and lymphoma are widely recognized, fewer people are familiar with the rare and serious condition myelofibrosis (MF).
Unlike fast-growing leukemias, MF typically progresses more slowly, but its impact on the body and blood cell production can be just as profound. The bone marrow of MF harbors a tangled web as complex as the origins of the disease itself, making it a challenging and enigmatic blood cancer.
Where does myelofibrosis fit among blood cancers?
Most blood cancers start in the bone marrow — the soft tissue inside bones where blood cells are made. While leukemia features an overproduction of abnormal white blood cells and multiple myeloma originates in plasma cells, MF belongs to a different category known as myeloproliferative neoplasms (MPNs). These diseases involve the bone marrow (myelo) producing excessive amounts (proliferative) of one or more blood cell types, which can lead to complications like clotting, bleeding, or fibrous scarring in the bone marrow.
MF disrupts the structure of bone marrow by replacing normal tissue with strands of scar-like fibrotic material. Doctors grade the hallmark scarring along a continuum, with higher scores associated with worse symptoms and outcomes. This scarring interferes with the marrow’s ability to produce healthy blood cells, causing dysfunctional blood cell production, an enlarged spleen, and even the potential progression to acute myeloid leukemia.
MF can develop independently as primary MF. However, it can also develop in patients with other MPNs, making it one of the rare blood malignancies that can appear as a secondary disease, secondary myelofibrosis.
Symptoms and disease progression
While some blood cancers cause rapid declines in health, MF has a highly variable course. Some people go years without a diagnosis, living with minimal symptoms, while others experience severe fatigue, weight loss, bone pain, blood clots, or the life-threatening progression to leukemia. MF can feature an enlarged spleen (splenomegaly) caused by dysfunctional blood cell production in the bone marrow, unlike leukemia, where swollen lymph nodes are more common.
MF can be difficult to diagnose because its symptoms overlap with those of other bone marrow diseases. Thorough diagnosing requires blood tests and a deep investigation into genetics and bone marrow structure. Doctors use a combination of blood tests for abnormal blood cell counts and genetic mutations, bone marrow biopsies to assess fibrosis levels, and, potentially, imaging scans to evaluate spleen and liver enlargement.
Unlike other blood cancers that require immediate chemotherapy, the treatment for MF is highly dependent on the patient’s risk factors, symptom severity, and disease progression. Doctors could recommend anything from a “wait-and-see” approach to cytoreductive therapies (such as JAK inhibitors or interferons). In advanced cases of MF, stem cell transplantation offers a potential cure, which must be balanced with serious risks. Unlike the well-established chemotherapy regimens of lymphoma and myeloma, MF treatment options are more limited but continue to expand with ongoing research.
Why understanding myelofibrosis matters
Blood cancers are highly complex, with overlapping symptoms and treatments. Research and discoveries in MF may also prove beneficial for patients with other types of blood cancers.
For those living with MF, organizations like the MPN Research Foundation provide access to valuable resources and updates on research and treatments. If you or a loved one is affected, visit mpnresearchfoundation.org to learn more and join our newsletter, where we report on the latest research.
