The spleen aids in the fight against infections by filtering out old or damaged blood cells. It also contains red blood cells and platelets, which aid in blood clotting.
When you have myelofibrosis (MF), bone marrow scarring causes decreased blood cell production. Blood cells are eventually created in other sections of your body, such as your spleen, outside of the bone marrow.
Extramedullary hematopoiesis is the term for this type of hematopoiesis. As the spleen works harder to produce these cells, it might grow excessively large.
Splenomegaly (enlargement of the spleen) can induce a variety of unpleasant symptoms. When it enlarges and presses up against other organs, it might cause abdominal pain and make you feel full even if you haven’t eaten much.
Tumors in Other Parts of Your Body
Noncancerous tumors of growing blood cells can arise in various parts of the body when blood cells are created outside of the bone marrow.
These tumors have the potential to induce gastrointestinal bleeding. You may cough or spit blood as a result of this. Tumors can potentially trigger seizures by compressing the spinal cord.
The portal vein transports blood from the spleen to the liver. High blood pressure in the portal vein is caused by increased blood flow to an enlarged spleen in MF.
Excess blood can be forced into the stomach and esophagus as blood pressure rises. This may induce bleeding by rupturing smaller veins. This problem affects about 7 percent of MF patients.
Low Platelet Counts
Platelets in the blood aid in the clotting of blood after an injury. As MF advances, platelet counts may drop below normal. The lack or fear of platelets is called thrombocytopenia.
Your blood won’t clot properly if you don’t have enough platelets. As a result, you may bleed more easily.
Bone and Joint Pain
MF can harden your bone marrow and lead to inflammation in the connective tissues around the bones. This leads to bone and joint pain.
The body produces more uric acid as a result of MF. When uric acid crystallizes, it can accumulate in the joints. Gout is the medical term for this condition. Gout can cause painful and inflamed joints.
Anemia, or a decreased red blood cell count, is a typical MF symptom. Anemia can become severe at times, resulting in debilitating exhaustion, bruising, and other symptoms.
Acute Myeloid Leukemia (AML)
About 15 to 20 percent of MF patients develop acute myeloid leukemia (AML), a more serious form of cancer. AML is a blood and bone marrow cancer that progresses quickly.
Treating MF Complications
To treat MF complications, your doctor may prescribe a variety of therapies. These include:
- JAK inhibitors, including ruxolitinib and fedratinib
- immunomodulatory drugs, such as thalidomide, lenalidomide, interferons, and pomalidomide (Pomalyst)
- corticosteroids, such as prednisone
- surgical removal of the spleen (splenectomy)
- androgen therapy
- chemotherapy drugs, such as hydroxyurea
Reducing Your Risk of MF Complications
Working with your doctor to treat MF is critical. To reduce the chance of MF problems, you should be monitored on a regular basis. Once or twice a year, or even once a week, your doctor may suggest that you come in for blood tests and physical exams.
There’s little evidence that earlier measures will help you if you have no symptoms and a low-risk MF. Your doctor may decide to wait until your condition worsens before starting treatment.
Your doctor may prescribe medications if you have symptoms or intermediate –
Ruxolitinib and fedratinib are JAK inhibitors that target abnormal pathway signaling caused by a common MF gene mutation. These medications have been demonstrated to decrease spleen size and treat other debilitating symptoms like bone and joint pain. They may considerably lower the risk of complications and increase survival, according to research.
A bone marrow transplant is the only treatment that can potentially cure MF. It involves receiving an infusion of stem cells from a healthy donor, which replace faulty stem cells causing MF symptoms.
This procedure carries significant and potentially life-threatening risks. It’s usually only recommended for younger patients without other preexisting health conditions.