Nearly 50 percent of people with myeloproliferative neoplasms (MPNs) experience headaches, which can coincide with other neurological symptoms such as fatigue, poor concentration, dizziness, and bone pain. MPNs are a group of rare progressive blood cancers that impact the production of blood cells.

People with MPNs may struggle to manage their symptoms, as myMPNteam members attest:

• “There is no rhythm or reason to this disease. Every day is different.”
• “It is affecting my quality of life so much right now.”
• “This year has been very challenging for me because of my MPN.”

There are three main types of MPNs:

• Essential thrombocythemia (ET), in which bone marrow produces too many platelets and results in a high platelet count, or a thrombocytosis
• Polycythemia vera (PV), in which there is an overproduction of red blood cells
• Myelofibrosis (MF), which causes severe scarring — or fibrosis — of the bone marrow

In the early stages of MPNs, some people are asymptomatic. Common symptoms like headaches, which typically occur in the general population, may be overlooked and delay diagnosis. A bone marrow biopsy or blood test that shows high levels of red blood cells, white blood cells, or platelets can indicate an MPN. Medical advances, including identifying mutations in the Janus kinase (JAK2) and the Calreticulin (CALR) genes have helped identify the disease and its characteristics. Headaches generally occur when the disease is active within capillaries, which are the body’s smallest blood vessels.

To learn more about headaches as a symptom of MPNs, myMPNteam spoke to Dr. Andrew Kuykendall, a physician at the Moffitt Cancer Center in Tampa, Florida. Dr. Kuykendall conducts clinical research to develop novel treatments for MPNs and is an active member of the MPN-Research Consortium.

What Causes Headaches in MPNs?

People with MPNs may experience secondary headaches, which are attributed to the disease or can be a side effect of treatment. Headaches that are unrelated to an underlying condition are known as primary headaches, and can occur among people with MPNs as well. Headaches that occur within three months of an MPN diagnosis are typically considered secondary symptoms of the disease.

Headaches Due To Thickened Blood

In ET, too many platelets and enlarged platelets can lead to increased interaction with blood vessel walls. An abnormal increase in platelets can create microvascular blockages in these blood vessels and cause symptoms such as headaches and dizziness.

Dr. Kuykendall noted that, with ET, microvascular symptoms are common. “With ET, what we see more commonly are headaches, as well as some of these microvascular symptoms, such as ringing in the ears, a feeling of fullness in the head, some numbness, tingling, and paresthesia [pins and needles sensation],” he said. “Luckily, many of these can be improved with just baby Aspirin [Acetylsalicylic acid].”

In PV, the production of too many red blood cells can thicken blood. On a blood test, this is a measurement called a hematocrit, which is the amount of someone’s blood that is made up of red blood cells. Thickened blood can reduce the flow of blood in capillaries and lead to headaches, blurred vision, and fatigue. Dr. Kuykendall noted that people with PV “at diagnosis often have hematocrits that are in excess of 55 percent or 60 percent.” At those levels, he and his colleagues will often see symptoms of facial redness, itching, and pressure-like headaches.

Headaches as Side Effects of Treatments

Drug therapies for MPNs can trigger headaches as a side effect, including:

• Janus kinase (JAK) inhibitors, such as Jafaki (Ruxolitinib), which targets JAK2 gene mutations
• Chemotherapy drugs, such as Hydrea (Hydroxycarbamide or Hydroxyurea), Interferon, or Agrylin (Anagrelide)
• Anemia drugs, such as Thalomid (Thalidomide) and Revlimid (Lenalidomide)

Bone Pain in the Skull

Abnormal production of blood cells in bone marrow and the scarring of bone marrow can create bone pain, particularly in MF. Headaches can occur when bone marrow in the skull is affected.

Primary Headaches

People with MPNs may experience primary headaches that are not symptomatic of an underlying disease. Some people may be genetically prone to primary headaches. They can also occur due to overactivity, chemical activity in the brain, muscle tension in the head or neck, alcohol, food additives, disrupted sleep, poor posture, hunger, and stress. People with MPNs should consult with their doctors to determine if their headaches are primary or secondary because the characteristics of both types of headaches can be similar.

Types of MPN Headaches

Headaches in people with MPNs can take various forms and may be of short or long duration. Dr. Richard Silver of Weill Cornell Medicine has observed persistent head pain more commonly in people with PV than with ET. But persistent headaches can occur in all types of MPNs.

Migraine-Like Headaches

Migraine headaches often occur with other symptoms, such as nausea, vomiting, sensitivity to light and sound, and visual auras or disturbances — such as blurred or fractured vision, shimmery lines, and blind spots. People with MPNs may experience headaches that are classified as migraines or that resemble migraines. A distinction between standard migraines and migraine-like headaches with MPNs is important because some medications used for standard migraines are not recommended for MPNs. For people with ET, migraine-like headaches tend to occur suddenly, whereas standard migraines occur gradually.

Silent Migraines

Migraine-like symptoms that may include visual disturbances, nausea, and sensitivity to light and sound, but no actual headache, are known as silent migraines. These represent another type of headache associated with MPNs, particularly ET and PV.

Cluster Headaches

Headaches that occur unilaterally, or on one side of the head, are known as cluster headaches and are generally considered a primary headache. Cluster headaches can induce intense eye pain and nasal congestion. Cluster-like headaches with PV have been documented, but rarely.

Treatment Options for Headaches With MPNs

MPNs are chronic diseases that are managed with a variety of treatments. The only potential cure for MPNs is stem cell transplantation, which has numerous risks and is not routinely recommended. Some MPN treatments can alleviate symptoms like headaches and should be discussed with your doctor.

Low-Dose Aspirin and Paracetamol

Low-dose Aspirin is generally recommended for low-risk cases of ET, PV, and MF. People with MPNs are at risk of blood clots, which increase the chance for strokes and heart attacks. Aspirin is known to reduce platelet function and can decrease blood viscosity and clotting. Some people with MPNs experience relief from headaches and visual disturbances with low-dose Aspirin. As Dr. Kuykendall noted, headaches and microvascular symptoms can improve with low-dose Aspirin.

Other over-the-counter (OTC) painkillers, such as Tylenol (Acetaminophen), are also used to treat headaches with MPNs, sometimes in combination with low-dose Aspirin.

Always consult your doctor before using new medications, even OTC remedies, to make sure there’s no risk of a dangerous interaction with your MPN treatment or other medications.

Phlebotomy

For people with low-risk PV, phlebotomy is a standard treatment in which blood is withdrawn to decrease the volume of the blood and of red blood cells. Phlebotomy lowers the volume of the blood and can have an immediate effect of reducing headaches and dizziness. In Dr. Kuykendall’s experience, once people living with PV “start to get phlebotomies or are able to get the hematocrits down with medications, they'll talk about how their headaches improve after each phlebotomy.”

But withdrawing blood can also deplete energy. As one myMPNteam member wrote, “I woke up tired and fatigued. My last phlebotomy wiped me right out. It took almost seven days to feel a little better.”

Many people with MPNs combine multiple treatments. One myMPNteam member described her regimen, noting, “I was just diagnosed with PV last month. I’m on daily Aspirin, a blood pressure pill three times a day, and phlebotomy every two weeks.”

Talk To Your Doctor About Headaches

If you are suffering from headaches due to MPNs, be sure to talk to your hematologist or oncologist about your symptoms. Your doctor can work with you to identify the cause of your headaches and find treatments that are compatible with other medications you may be taking.

You Are Not Alone: Finding Support for Headaches With MPNs

Join others with MPNs on myMPNteam to discuss symptoms, treatments, and side effects. The MPN community is growing, and people with MPNs are sharing valuable information and experiences. Managing an array of symptoms, including headaches, is a challenge for people with MPNs, but sharing experiences and knowledge can help.

Here are some myMPNteam conversations you may want to join:

• “Has anybody else had upper stomach and back pain? With headache and nausea?"
• “I have headaches, body aches, itching, fatigue."
• “I have not needed a phlebotomy in about three weeks, but feeling the head fullness."

What are your experiences with MPNs and headaches? How do you manage your symptoms, and what tips do you have for other people with MPNs? Which treatments work best for you? Share your comments below or go to myMPNteam and start a conversation today.