What are the Symptoms of Myeloproliferative Neoplasms (MPNs)?

General symptoms of MPN include the following:

• Itching
• Fever and night sweats
• Fatigue
• Bone pain
• Spleen enlargement and its complications
• Headaches and ringing in the ear

Itching

One of the most common symptoms of MPNs is itching. Itching may be referred to as pruritis by your healthcare team. In MPN, cytokines (specialized proteins) within the cell signal inflammation, which results in an itching sensation. In some forms of MPN, patients may experience aquagenic pruritis, which is a type of itch that occurs once the body has come into contact with water. 

The National Comprehensive Cancer Network (NCCN) recommends that patients who experience itching should first try to incorporate sensitive skin care routines into daily living. Adjusting to a shorter shower schedule and using a mild soap may help to alleviate associated symptoms. 

If incorporating sensitive skin routines into daily care does not help, your provider may suggest utilizing an over-the-counter antihistamine (ex: diphenhydramine or cetirizine) or may prescribe a topical steroid treatment to apply on affected areas.  

Fever and Night Sweats

Similarly, to itching, cytokines within the blood can also signal inflammatory responses within the body that can result in fever or night sweats. Fevers are generally low-grade and can be controlled/reduced over time with certain medications. However, it is important to monitor for fevers and let your healthcare team know the duration and frequency of fever episodes. 

According to a peer-reviewed study in the journal Leukemia, patients diagnosed with MPNs are at an increased risk for infection. As a result, your provider will want to know if you are experiencing fevers to determine if the fever is due to the underlying MPN cancer or instead caused by an emerging infection. 

To help manage the symptoms of night sweats, the National Cancer Institute explains learning relaxation exercises can help patients decrease the perceived burden. Examples of relaxation exercises include yoga, meditation, and slow breathing. Changing bedding out to lighter, breathable sheets and having a cool glass of water near the bedside table may also help. 

Fatigue

Feeling tired (fatigue) is consistently recognized as a common symptom of MPNs. In a survey conducted on patients with myelofibrosis, polycythemia vera, and essential thrombocytopenia, fatigue was reported as the top symptom that patients wanted resolved. 

Fatigue can be a result of anemia, a condition that occurs when there is not enough oxygen in your bloodstream. For those with certain myeloproliferative neoplasms, this may occur due to the overall decrease in the number of normal healthy red blood cells that results when the body is overproducing a different type of blood cell. Examples of MPNs that may accompany fatigue as a result of anemia include:

• Primary Myelofibrosis
• Chronic Myeloid Leukemia
• Chronic Neutrophilic Anemia
• Polycythemia Vera

The exact cause of fatigue across other MPNs is unknown, although it is thought to be due to comorbid health conditions and medications being utilized, among other reasons. The MPN Research Foundation suggests taking short naps during the day and incorporating moderate exercise into your daily routine can help to alleviate fatigue over time. 

Bone Pain

Many patients with myeloproliferative neoplasms experience bone pain. Bone marrow is the spongy substance within the middle of our bones and is responsible for creating stem cells that eventually become white blood cells, red blood cells, and platelets. Because there is an overproduction of certain blood cells occurring in MPNs, the bone marrow can sometimes extend out further than the center of the bone within long bones, resulting in unwanted inflammation, pressure, and pain. 

Antihistamines (ex: loratadine) and anti-inflammatory medications (ex: naproxen) have been commonly used to treat initial bone pain. In some instances, your provider may recommend stronger medications or single-fraction radiation for temporary bone pain relief. 

Symptoms due to Spleen Enlargement

Because myeloproliferative neoplasms cause an increase in blood cells, they can also impact your spleen. The spleen is an organ located inside the rib cage above your stomach that filters and creates certain types of blood cells. Whenever more blood cells are in the bloodstream, there are more blood cells running through the spleen, which can result in a condition called splenomegaly (or enlargement of the spleen). 

Whenever the spleen becomes enlarged, it can push up against other organs within your body. This pressure can cause overall abdominal discomfort. Furthermore, if the spleen is pushing against the stomach, it can cause patients to feel full more quickly. 

Symptoms related to spleen enlargement can be resolved with reducing the spleen size. Many medications and treatments may work to help shrink spleen size. Examples include, but are not limited to, the following: 

• Hydroxyurea (Hydrea)
• Ruxolitinib (Jakafi)
• Interferon alfa (e.g., Intron-A, Roferon-A)
• Surgical splenectomy

Headache/Ringing in the Ears

Headaches and ringing in the ears are also reported side effects of MPNs. These phenomena are generally caused by either a decrease in blood flow to the brain or due to an increase in platelets within the blood stream. 

If you experience headache or ringing in the ears, it is important to discuss the symptoms with your healthcare team. Although these symptoms are common for people with MPNs, they can also be a sign of a stroke or other thrombotic event that can occur. As a result, your provider will want to closely evaluate you to assess for any new thrombosis (blood clots). 

Common headaches may be relieved using over-the counter pain relievers. Furthermore, migraine headaches associated with MPNs can also be treated by prescription with a class of medications called triptans (ex: sumatriptan, rizatriptan, etc.). These medications can work to relieve migraine headaches at the beginning of a migraine episode. 

Other Symptoms

Other symptoms related to individual myeloproliferative neoplasms include: 

• Liver enlargement (hepatomegaly) in primary myelofibrosis
• Cold fingers or toes in essential thrombocythemia 
• Gout outbreaks in chronic neutrophilic leukemia
• Angioedema or numbness or tingling in hands and feet in chronic eosinophilic leukemia

Tracking Your MPNs Cancer Symptoms

Regardless if you are actively being treated for your myeloproliferative neoplasm, it is important to track any changes in your physical, mental, or emotional health. New or worsening symptoms may indicate that treatment is needed, current treatment is no longer effective, or that the disease has progressed. 

Certain companies, like Voices of MPN, have created an MPN toolkit available for patients, complete with a symptom tracker to better document potential associated symptoms. Your healthcare team may also recommend other symptom trackers available unique to the myeloproliferative neoplasm you are diagnosed with. 

Moving Forward – Questions to Ask Your Doctor

• How should I track potential symptoms related to my MPNs?
• Are there treatments to help relieve my symptoms?
• What should I do if my symptoms are becoming worse?

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