What is myelofibrosis?
Myelofibrosis as it turns out is actually a chronic blood cancer where one of the stem cells in your body gets a mutation and that mutation leads to increased inflammation created by those cells, which can ultimately cause scarring in the bone marrow called fibrosis.
If we think about it in terms of having a garden where the seeds are your early blood cells, one of those seeds gets a mutation over time. There are weeds, and those weeds can kind of crowd out the rest of the blood cells. It can also poison the soil, making the soil not so great for the other blood cells to grow.
And it also turns out that those seeds can travel, the body and leave the bone marrow and lodge in places that also have soil like the spleen, where they can make the spleen really enlarged. And can cause symptoms of spleen majorly, which can include right upper or left upper quadrant pain and feeling full after just a few bites of food.
In this particular disease, what happens is the bone marrow overproduces cells. And usually cells that we think of as megakaryocytes are atypical platelet precursors. These cells are very fibrotic. And they produce things that we think of that we call inflammatory cytokines. And this leads to the development of fibrosis or scarring within the body's bone marrow or the body's blood factory.
And so over time, patients are less able to make blood in their blood factory because of the deposition of this type of scarring.