When you’re first diagnosed with primary myelofibrosis (PMF), you may not experience any symptoms. Your doctor or hematologist may take a “watch-and-wait” approach, monitoring your condition for evidence that it’s progressing. Between follow-up appointments, you may be left wondering how you’ll know if something’s wrong.

In this article, we’ll go over six signs that may mean your PMF is progressing, so you’ll be ready to make an appointment with your doctor or hematologist as soon as possible for testing. Fast treatment is the best way to slow the progression and give you the best chance for a good outcome.

How Does Primary Myelofibrosis Progress?

PMF is a type of myeloproliferative neoplasm (MPN) that develops in your bone marrow — the spongy tissue inside your bones that makes new, healthy blood cells. Normally, bone marrow cells mature and develop into red blood cells, white blood cells, and platelets.

In PMF, your blood cells don’t mature properly, leading to a surplus of immature blood cells known as blasts. The collagen fibers inside your bone marrow become extremely thick and look almost like scar tissue. These fibers fill the inside of the bone, making it difficult for bone marrow cells to develop into healthy blood cells.

Because your bone marrow can’t function properly, your liver and spleen try to pick up the slack and make new cells. In the early PMF stages, you likely won’t notice any new symptoms because the condition progresses slowly over time. Your doctor may see changes in your blood cell counts during a routine blood test. Most commonly, your red blood cells will be low, while your white blood cell and platelet counts will either be high or low, depending on your disease status.

Most PMF cases aren’t treated until symptoms appear. It’s important to know what signs to look for so you can start treatment as quickly as possible. Here are six signs that may mean your PMF is progressing.

1. General Weakness and Tiredness

PMF typically causes anemia (low red blood cell counts). Your red blood cells use the protein hemoglobin to carry oxygen throughout your body. Your tissues and organs need oxygen for energy — without enough oxygen, your body won’t have enough energy to perform essential functions.

The most common symptoms of anemia are weakness and fatigue. You may find yourself struggling to get through the day with low energy levels. Members of myMPNteam often write about how fatigue affects them. “Family just don’t understand how tired this condition makes you,” shared one member.

“I’m tired of being tired,” wrote another.

You may also experience:

• Dizziness or light-headedness that makes you feel uneasy or unsteady
• Shortness of breath, or the feeling that you can’t breathe deeply or catch your breath
• Pain, squeezing, or a feeling of pressure on your chest
• Arrhythmia (a fast, racing, or skipping heartbeat)
• Pulsatile tinnitus (a whooshing or pounding sound in one ear that comes and goes over time)

Learn about ways to manage fatigue.

2. Easy Bruising or Bleeding

Have you noticed more bruises on your arms or legs lately? Or do your gums now bleed when you’re brushing your teeth? This may suggest that your PMF is progressing. In addition to causing low red blood cell counts, PMF can lead to thrombocytopenia — a low platelet count.

Platelets are tiny cell fragments in your blood that help form blood clots. They work together with other proteins and blood cells to plug cuts, scrapes, and other injuries and stop bleeding. If you have thrombocytopenia, you don’t have enough platelets to properly clot your blood.

Symptoms of thrombocytopenia include:

• Bruising or pooling of blood underneath your skin
• Frequent nosebleeds
• Bleeding or swollen gums
• Blood in your urine or stool
• Heavy menstrual periods
• Petechiae (tiny, rashlike red or purple dots on your lower legs)

Some myMPNteam members share their worries about these symptoms. “I had a very scary nosebleed,” wrote one member. “Now I’m feeling dizzy and very tired.”

“I’m not so good today,” another said. “Recently, I’ve noticed bruises, one under my foot and then another few randomly. They seem to be on veins, so it’s a bit scary!”

Read more about how MPNs affect blood cell counts.

3. Eating Less Than Usual Due to Reduced Appetite

You sit down for a delicious dinner but find yourself feeling full after just a few bites. When living with PMF, this symptom is cause for concern. The likely culprit is splenomegaly — an enlarged spleen.

Your spleen is located on your left side, above your stomach, and inside your rib cage. It helps filter your blood and makes new white blood cells to fight infections. It’s also responsible for storing red blood cells and platelets.

In PMF, your bone marrow can’t keep up with demands for new, healthy blood cells. Your spleen steps up to the plate and starts making cells. As a result, your spleen grows and starts pressing on nearby organs — like your stomach. This is why you feel full even when you haven’t eaten much. You may also experience some pain and discomfort in your upper left abdomen.

One member shared their experience of spleen problems with MPNs: “They put me on hydroxyurea after my spleen became so enlarged I felt like a bloated whale if I ate or drank.”

Splenomegaly is most common in PMF, but it can also occur in people with essential thrombocythemia and polycythemia vera, other types of MPNs. If you’re feeling too full too fast or experiencing upper abdominal pain, it’s time to talk to your doctor.

4. Excessive Night Sweats and Unexplained Weight Loss

Waking up in the middle of the night is never good, but it’s even worse if you wake up drenched in sweat. While some sweating during the night is normal, it’s cause for concern if you’re soaking through your bedsheets and pajamas most nights.

Night sweats are common among myMPNteam members, who have described their experiences:

• “I sweat up my night shirts every night.”
• “I wake every day or during the night drenched in sweat.”
• “I have drenching night sweats. I call them SINS: sleep-interrupting night sweats.”

Excessive night sweats are another sign of MPNs, and they’re common in people with PMF. This disease causes body-wide inflammation that leads to fevers and night sweats.

As your PMF progresses, you may also begin to lose weight without trying. This seemingly unexplained weight loss happens because your body is using lots of energy to fuel inflammation and cancer cell growth.

5. Frequent Infections

Your white blood cells are part of your body’s defense system against invading bacteria, viruses, and fungi. Your bone marrow is responsible for producing new, healthy white blood cells. Most people with PMF make too many, but these white blood cells don’t work as well as they should.

Without proper protection, you’re more likely to get sick. Frequent bacterial or fungal infections indicate that your PMF is progressing.

6. Bone or Joint Pain

PMF affects your bone marrow, so it makes sense that you may experience bone or joint pain as your disease progresses. While doctors and researchers aren’t quite sure why bone pain occurs, they think it may be due to:

• Overgrowth of the bone marrow
• Osteosclerosis (bone hardening)
• Periostitis (inflammation of the connecting tissue supporting the bones)

PMF also boosts your body’s production of uric acid, a waste product made from breaking down certain foods and alcohol. Too much uric acid can lead to an inflammatory joint condition known as gout. Uric acid crystals form in the joints, causing sharp pain and swelling.

Several myMPNteam members have discussed their experiences with gout. “My doctor checks my uric acid levels with every blood work since myelofibrosis can cause gout,” shared one member. “It’s very painful and can be red and swollen also.”

“I had some gout in my right knee a few days ago, but it seems to have gotten better lately,” another member said.

Talk to Your Doctor About Your New Symptoms

If you’ve started experiencing one or more of these PMF symptoms, it’s time to check with your doctor. They can run extra tests and determine if your disease is progressing. It’s vital to start treatment as soon as possible if your PMF is transforming into acute myeloid leukemia. This much more aggressive type of blood cancer takes on new mutations and is difficult to treat.

Treatments for Primary Myelofibrosis

Several treatment options can manage anemia from PMF. Immunomodulators help fight cancer cells and clear room in your bone marrow for healthy blood cells. Examples include lenalidomide (Revlimid) and thalidomide (Thalomid). You may also receive a blood transfusion with red blood cells from a healthy donor to temporarily relieve your symptoms.

If your doctor or hematologist finds PMF progression after you report night sweats or weight loss, they may prescribe you anti-inflammatory medication. One example is prednisone, which belongs to a group of drugs called corticosteroids (steroids). Prednisone reduces inflammation throughout the body.

Janus kinase (JAK) inhibitors are another PMF treatment that reduces inflammation. Specifically, JAK inhibitors block pathways in cancer cells. Examples of these medications include:

• Ruxolitinib (Jakafi)
• Pacritinib (Vonjo)
• Fedratinib (Inrebic)

“Watching and waiting” doesn’t mean doing nothing. By recognizing the signs of PMF progression and alerting your doctor right away, you can begin any needed treatment as soon as possible.

Talk With Others Who Understand

Find others to connect with on myMPNteam, the online social network for people and their loved ones living with myeloproliferative neoplasms. On myMPNteam, more than 3,600 members come together to ask questions, give advice, and share their stories with others who understand life with MPNs.

Have you experienced symptoms that suggest your myelofibrosis is progressing? Which symptoms appeared first? Share your experience in the comments below, or start a conversation by posting on your Activities page.