Can Myelofibrosis Go Away On Its Own?

Can Myelofibrosis Go Away On Its Own?

No, myelofibrosis or primary myelofibrosis (PMF) does not go away on its own. It is a bone marrow disorder due to a gene mutation in the stem cells which leads to the production of abnormal cells and scar tissue formation in the bone marrow. Therefore, PMF does not cure or go away on its own; there is an 8-23%risk of PMF progressing to acute leukemia (commonly acute myeloid leukemia) within 10 years after the diagnosis.

First, let us see what is the cause of primary myelofibrosis, and then you will understand why we are telling it does not go away on its own.

Can Myelofibrosis Go Away On Its Own?

What Leads To Myelofibrosis?

The exact cause of primary myelofibrosis is not clear but it might be associated with several gene mutations such as JAK-2, CALR, and MPL. JAK-2 mutation is seen in 50% of patients diagnosed with primary myelofibrosis, the mutation is also present in other associated disorders with primary myelofibrosis which are polycythemia vera and essential thrombocytopenia. The CALR mutation is seen in 20% of patients and MPL mutation in 10% of patients with primary myelofibrosis. There are many other possible mutations as well. But how exactly these mutations occur in the first place is still not known, it is believed to be an acquired mutation. There might be a chance that not just one mutation but several mutations together give rise to primary myelofibrosis.

The mutation occurs in one stem cell then this stem cell multiplies along with the mutation and as a result, abnormal cells and scar tissue is formed in the bone marrow. With time these abnormal cells outnumber the normal blood cells and the scarring affects the function of the bone marrow further. The scarring mainly occurs due to an increase in the platelet precursor cells megakaryocytes which lay fibroblasts that form scar tissue. Bone marrow function is affected severely which results in reduced red blood cells, white blood cells, and platelets. Therefore, organs such as liver and spleen are stimulated to make blood cells; as a result, these organs enlarge. The symptoms are due to these occurrences.(1)

There are several other theories regarding the origin of primary myelofibrosis such as

Previous exposure to toxic substances such as benzene, phosphorous and fluoride and tuberculosis infection might cause primary myelofibrosis.

Primary myelofibrosis in the bone marrow can be a metastasis of a primary tumour and common primary tumors that can metastasize are breast, lung, prostate, thyroid gland, adrenal gland, or kidney.

Primary myelofibrosis can be a secondary characteristic of another bone marrow disease such as chronic myeloid leukemia, polycythemia vera, multiple myeloma, and metabolic disorders.

As you understand primary myelofibrosis is not reversible, it is a progressive disease eventually the bone marrow fails completely; there is a high risk of primary myelofibrosis progressing to acute leukemia. Most patients are asymptomatic in the initial stages until the bone marrow fails to a significant stage and symptoms due to enlarging liver and spleen occur. Allogeneic stem cell transplantation is the only potential cure but it is only offered to younger patients with advanced disease and compatible donor. Primary myelofibrosis is common in elderly therefore; many patients are not suitable candidates for transplantation. Some patients remain symptom-free for longer periods even after diagnosis and until symptoms appear treatment is not started but the patient is regularly evaluated. When symptoms appear, treatment is definitely needed and it is focused on relieving symptoms.(2)

Conclusion

No, primary myelofibrosis does not go away on its own; some patients might remain symptoms free even after the diagnosis of primary myelofibrosis. That does not mean the disease is cured on its own as symptoms usually appear after some time and during this period watchful waiting is important so, that treatment can be initiated as soon as the condition starts getting bad. There is also an 8-23% risk of primary myelofibrosis progressing to acute leukemia within the first 10 years after diagnosis. The only potential cure is allogeneic stem cell transplantation but it is not offered for all patients as there can be life-threatening complications. So, treatment is focused mainly on relieving symptoms.

References

  1. https://www.leukaemia.org.au/disease-information/myeloproliferative-disorders/types-of-mpn/primary-myelofibrosis/
  2. Prognosis of primary myelofibrosis in the genomic era https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4987499/

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