Myelofibrosisis a rare jot down of bone marrow cancer.
This article will explain the symptoms of myelofibrosis, how it is diagnosed, and how it is treated.
It is normally a soft, spongy texture.
Patricio Nahuelhual / Getty Images
It producesWBCs,RBCs, andplatelets.
Eventually, abnormal cells are present in high enough numbers to crowd out the healthy cells.
Myelofibrosis Types
The initial cause of abnormal bone marrow development determines the bang out of myelofibrosis.
The two types are primary and secondary.
Primary Myelofibrosis
With primary myelofibrosis, the change in the bone marrow cells happens spontaneously.
It doesnt occur due to a previous bone marrow condition.
Myelofibrosis Symptoms
As too few blood cells are made, symptoms will start to develop.
This mutation isn’t inherited.
Instead, it develops spontaneously in a bone marrow cell.
It produces a protein that causes the bone marrow to overproduce platelet precursor cells calledmegakaryocytes.
Other gene mutations that may play a role in developing myelofibrosis include the CALR and MPL genes.
During this evaluation, a healthcare provider may start with a detailed history and physical examination.
Blood work may be taken which can start the process of finding a diagnosis.
Abnormal findings in the CBC may lead to further testing, which may include a bone marrow biopsy.
During abone marrow biopsy, a small sample of bone marrow is taken, often from the hip.
This test can result in a diagnosis of myelofibrosis.
The higher the score, the more high-risk their myelofibrosis is.
If someone is experiencing symptomaticanemia(low RBCs) because of myelofibrosis, they may receive periodic RBCtransfusions.
There are additional medications that may be given to help the bone marrow make red blood cells.
This may not completely resolve anemia but can keep the red blood cells up at a tolerable level.
If someone also has iron deficiency anemia, iron supplements may help improve red blood cell levels.
To reduce high levels of WBCs and platelets, medications to suppress the bone marrow may be given.
An example of one of these medications is hydroxyurea.
This can be done through radiation to the spleen or bysurgical removal of the spleen.
This medication interferes with the JAK2 pathway that the cells use to grow.
Can Myelofibrosis Be Cured?
The majority of cases of myelofibrosis are treated with the goal of decreasing symptoms of the disease.
An attempt can be made to cure the disease through astem cell transplant.
This approach requires large doses of chemotherapy to kill all of the cancer cells.
Stem cells are collected before the procedure to be infused back in after chemotherapy has worked.
These stem cells can then begin to resume making normal WBCs, RBCs, and platelets.
This procedure is not recommended for everyone with myelofibrosis, as it can lead to severe complications.
As the disease progresses and the blood counts continue to decrease, complications may arise.
With the decrease of white blood cells comes a higher risk of developing infection.
Infections can occur anywhere in the body, though most often in the lungs.
The infection can be due to bacteria, viruses, or fungi.
With infection may come fever, increased weakness, and cough.
Not having enough normal platelets can lead to severe bleeding or hemorrhaging.
The bleeding can occur following an injury or can occur spontaneously.
The bleeding can become life-threatening if severe and not stopped quickly.
Blood clotting, the opposite of bleeding, could also occur.
This can lead to damage to the brain, heart, lungs, and extremities.
Transformation toacute myeloid leukemiaoccurs in 5% to 10% of those diagnosed with myelofibrosis.
Myelofibrosis Prognosis
The prognosis of myelofibrosis can vary from person to person.
It depends upon the pop in and risk category of their disease.
Prognosis can differ slightly based on which scale is used at the time of diagnosis.
Many of these are also associated with other conditions.
A workup and diagnosis can ensure you are getting the appropriate treatment.
Your provider may want to run additional tests or start treatment if your symptoms continue.
If severe symptoms develop, notify your healthcare provider immediately or seek emergency care.
Treatments are individualized, ranging from watchful waiting to stem cell transplant.
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