If your doctor has told you that you have myelofibrosis (MF), you probably want to learn more about your diagnosis and how it can affect you. Here’s a closer look at the condition, including other health problems it can trigger.
How Do You Get MF?
Researchers are still figuring out what causes MF, but there may be certain factors that increase your risk. One is a family of proteins called Janus kinases (JAKs). They send signals that impact how your body makes blood cells inside your bone marrow. And they work to control how many red blood cells, white blood cells, and platelets you have. When JAKs are doing their job right, they help your body make the correct number of blood cells. But if these proteins send too many signals, it leads to too many blood cells in your bone marrow.
When a mutated and overactive blood stem cell divides and makes copies of itself, it grows out of control. The result is the formation of many abnormal cells that make platelets (called megakaryocytes) in the bone marrow.
These abnormal cells can change the bone marrow by releasing signaling proteins that help control inflammation in your body (called cytokines). Some scientists think this might trigger the buildup of fibrous tissue in your bone marrow, which then thickens, resembling scar tissue. It eventually hinders the bone marrow from making normal blood cells, leading to MF symptoms and other health problems.
MF and Anemia
One health problem that happens a lot when you have MF is anemia. Anemia happens when you have too few healthy red blood cells to carry oxygen to your body’s organs. The condition may cause you to feel cold, tired, and weak. Without treatment, anemia can be fatal.
There are many causes of anemia, such as bleeding and a lack of iron, B12, or folic acid. Your doctor will figure out what’s causing your anemia before you start treatment. If you have MF, they may suggest a blood transfusion to treat your anemia. It can boost your red blood cells and improve your symptoms.
Additional anemia treatments
If your serum erythropoietin (EPO) levels (which show your amount of healthy red blood cells) have dropped below 500 mU/mL, you could need further treatment with a substance called an erythropoietin stimulating agent (ESA). Normally, your kidneys make erythropoietin, which triggers the bone marrow to make red blood cells. ESAs do the same thing, except scientists make them in a lab.
For people with MF whose serum EPO levels have risen above 500 mU/mL, treatment may include:
- Hormones. Synthetic male hormones called androgens can help your body make more red blood cells.
- Immunomodulators (IMiDs). These drugs can give your red blood cell counts a boost. You may take them with the steroid prednisone.
MF and Enlarged Spleen
MF often causes your spleen to grow larger, called splenomegaly, which can leave you with pain in your abdomen and under your left ribs. You’ll also feel full even on an empty stomach or after eating very little. An enlarged spleen could also cause other health problems, such as more pressure on blood flowing into your liver. This can cause the blood pressure in the portal vein to be too high, which is called portal hypertension.
Doctors treat an enlarged spleen with:
- Medication. Prescription drugs can shrink or control the size of your spleen and ease painful symptoms.
- Surgery. If other treatments don’t work, your doctor may suggest surgery to remove your spleen (called a splenectomy). While surgery can soothe your symptoms, it comes with a higher chance of bleeding, blood clots, infection, an enlarged liver, and a higher platelet count.
- Radiation therapy. This treatment uses high-powered X-rays to reduce the size of your spleen. It’s an option when other treatments haven’t worked or you’re unable to have surgery.
- Embolization. This is when a doctor injects a substance into your artery (through a catheter) to stop blood from flowing through your spleen to shrink it.
MF and Low Platelet Counts
A low blood platelet count, called thrombocytopenia, is common in people living with MF. With this condition, you’ll only be able to get certain treatments for MF. Another drawback is that an MF treatment that uses JAK inhibitors can lead to a drop in your platelet count. To treat thrombocytopenia, your doctor will closely watch you and possibly change your medication dose.
MF, Thrombocytosis, and Leukocytosis
With these conditions, your bone marrow makes too many platelets (thrombocytosis) or too many white blood cells (leukocytosis).
Treatment for thrombocytosis and leukocytosis involves a chemotherapy drug called hydroxyurea, which lowers high platelet and white blood cell counts. It could also ease other symptoms such as weight loss and night sweats while reducing the size of your enlarged spleen. You won’t be able to take this drug if you have low blood cell counts or severe anemia.
Questions for Your Doctor
An MF diagnosis can mean lots of questions for your doctor and the rest of your care team. Here are some questions to consider:
- What kinds of tests do I need?
- Is my condition likely temporary or lifelong?
- What treatment do you suggest?
- Are there alternative treatments?
- How can I best manage my other health conditions with myelofibrosis?
- Should I see a specialist?
- What should I read for more information about myelofibrosis?
Myelofibrosis Resources
Many resources are available to help you navigate the journey of living with myelofibrosis, from support groups to treatment and clinical trial information. Here are some that may be helpful:
- MPN Advocacy and Education International
- MPN Cancer Connection
- MPN Education Foundation
- MPN Research Foundation
- National Organization for Rare Disorders (NORD)
- Patient Power
- PV Reporter
Show Sources
Photo Credit: The Image Bank/Getty Images
SOURCES:
Leukemia & Lymphoma Society: “Myelofibrosis (MF),” “Support Resources.”
Cleveland Clinic: “Cytokines,” “Anemia,” “Erythropoietin.”
Mayo Clinic: “Myelofibrosis.”
Annals of Hematology: “Managing patients with myelofibrosis and low platelet counts.”
