What Is Myeloproliferative Neoplasm?

Myeloproliferative neoplasm is one of the rare blood cancers that start in your bone marrow when it starts creating too many platelets, white blood cells, or red blood cells. These blood cells are present inside the bones as spongy tissues. When you have MPN, the production process of blood cells suffers a lot.

Myeloproliferative neoplasm has a slow growth rate, which can go undetected for several years before you experience any of its symptoms. Other than that, this cancer has different names, such as myeloproliferative disorders and chronic myeloproliferative neoplasm. Generally, this cancer is not fatal, as it rarely becomes severe. However, if left untreated for a long period, this cancer may take a serious turn.

Another thing you should know about myeloproliferative neoplasm is that this cancer is not curable. But, your healthcare provider can treat the cancer symptoms and lower its risk of turning into a more severe condition. Myeloproliferative neoplasm ICD 10 code D4.7 is for chronic myeloproliferative disease. It comes under the range of Neoplasms, which WHO lists.

Understanding Different Types Of Myeloproliferative Neoplasms

Now, as mentioned, there are various types of MPN. The most common types of this cancer include polycythemia vera, essential thrombocythemia, and myelofibrosis. Furthermore, it also has less common types, which we discuss as we move further in this section. So, in general, there are seven types of myeloproliferative neoplasms.

The severity of your condition will vary based on what type of MPN you have. So, let’s understand each of these cancer types one by one.

Polycythemia Vera

Starting with MPN types, the first one we have is polycythemia vera, known to be the most common type of this cancer. In this condition, your bone marrow starts producing many red blood cells. And because of this, with too many red blood cells, your blood gets thick. This results in your blood moving slowly via the bloodstream.

People with polycythemia vera are more prone to forming blood clots, which may lead to stroke and heart attack. In rare cases, this cancer may progress to become an extreme blood condition, such as acute leukemia.

Essential Thrombocythemia

This condition occurs when the bone marrow produces too many platelets. If your blood vessel ruptures, platelets create blood clots that help stop or slow the bleeding. However, when you have essential thrombocythemia, the bone marrow produces platelets even when no blood vessel ruptures are present or need to be produced. And because of these too many platelets, you experience blood clots that increase the risk of a stroke or heart attack.

However, this MPN type has a slow progress rate. Usually, people learn about this condition if their blood tests display high levels of platelets. In rare cases, individuals having this cancer may develop leukemia.

Myelofibrosis

Myelofibrosis is an aggressive type of MPN. In this condition, the bone marrow makes unusual stem cells that turn inflamed and create scar tissue. With time, the bone marrow gets filled with scar tissue.

These scar tissues prevent the bone marrow from creating enough red blood cells, so less oxygen is passed throughout the body. Because of this, a person may get anemia. 

Furthermore, this condition also prevents your bone marrow from making sufficient platelets. So, while you have this, you will struggle to stop or slow down excessive bleeding. In rare cases, individuals with myelofibrosis may get acute myeloid leukemia.

So, after knowing the common types of myeloproliferative neoplasm, let’s look at some less common types of this cancer.

Chronic Eosinophilic Leukemia

In this cancer type, your bone marrow produces too many white cells, known as eosinophils. Just like the previous cancers, this cancer has a slow progression rate. In a few cases, this cancer can develop into acute myeloid leukemia. Another name for CEL is hypereosinophilic syndrome.

Chronic Neutrophilic Leukemia

In this cancer type, your bone marrow produces too many white cells, known as neutrophils.

Chronic Myelogenous Leukemia

In this cancer type, your bone marrow produces too many white cells, known as granulocytes. Over time, these cells start accumulating, which makes it difficult for the bone marrow to produce different blood cells in the body.

Myeloproliferative Neoplasm, Unclassifiable (MPN-U)

It is an MPN type that doesn’t fit into any other categories of this cancer. In this cancer type, your bone marrow produces too many different blood cells, such as red blood cells, platelets, or white blood cells.

How Does Myeloproliferative Neoplasm Affect Your Body?

Before we jump into learning about the various symptoms of myeloproliferative neoplasm types, it’s vital that you understand how this cancer affects the body. As previously mentioned, this blood cancer occurs due to the production of extra blood cells, which is more than your body can handle. And this impact can vary depending on the type of MPN cancer you have.

However, if you want to understand how myeloproliferative neoplasm affects your body, it would help to know about blood cell production and bone marrow first. The blood cells in your body start as stem cells in the bone marrow. And this bone marrow is found in the center of the bones and has a soft, sponge-like structure. This tissue produces cells that may become lymphoid or myeloid stem cells.

The lymphoid stem cells in your body turn into white blood cells that are there to fight infection. Meanwhile, the myeloid stem cells turn into red cells that are responsible for carrying oxygen throughout the body. The platelets in your body help prevent excessive bleeding.

Your genes offer the instructions for which your cells take form and function. Typically, the bone marrow makes stem cells that further multiply per the body’s needs. And the genes give these stem cells directions that they follow that aid in regulating cell formation.

Now, sometimes your genes get mutated. Because of this mutation, a few stem cells get new instructions from these genes to keep multiplying and dividing. Over time, these stem cells become blood cells, increasing in number and affecting blood flow. And having blocked blood flow can lead to multiple serious medical conditions.

What Are Myeloproliferative Neoplasm Symptoms?

In the early stages of MPN, generally, there are no symptoms. So, you may not know you have this cancer unless you’re going for regular blood tests. However, splenomegaly may be the first sign that you’ll notice as your cancer progresses. Also known as an enlarged spleen, during this, you may experience fullness, discomfort, or pressure below the ribs on the left side. This is where you can find the spleen.

This is one of the most common signs of different MPNs. However, this symptom is less common if we talk about essential thrombocytopenia. The symptoms you may face during this cancer can vary based on what type of cancer you have.

Chronic Eosinophilic Leukemia

A common symptom of chronic eosinophilic leukemia israshes. Other than that, you may feel feverish or tired. Furthermore, the other symptoms that occur due to this condition may vary based on the affected parts of your body.

Chronic Neutrophilic Leukemia And Chronic Myelogenous Leukemia

The symptoms of this cancer are as follows:

• Fatigue and fever
• Unexpected weight loss and loss of appetite
• Bone pain
• Bruising easily
• Night sweats

Polycythemia Vera

The symptoms of this cancer are as follows:

• Fatigue
• Headaches
• Double or blurred vision
• Dizziness

Essential Thrombocythemia

The symptoms of this cancer are as follows:

• Bleeding from the intestines or stomach
• Bruising easily
• Blood in the pee
• Unexplained bleeding or bruising from the gums, nose, and mouth

Primary Myelofibrosis

In this condition, a person may face anemia symptoms such as weakness, fatigue, and dyspnea. Other symptoms may include:

• Itchy skin
• Bone pain
• Pale skin
• Weight loss
• Night sweats
• Early satiety or Abdominal fullness
• Fevers

What Are Myeloproliferative Neoplasm Causes? 

Now, we are aware of what the common symptoms are for different types of myeloproliferative neoplasm. So, the next thing we should know is the causes of MPN. However, before we discuss the causes, one thing you should understand is acquired genetic disorders. Now, this refers to conditions that are caused due to acquired mutations or changes in DNA parts. And MPNs come under acquired genetic disorders.

Because these cancers are acquired genetic disorders, there is almost no chance you’ll inherit these conditions from your parents. As per the reports of medical researchers, the following are the genetic mutations that may lead to MPN:

Mutations Associated With Janus Kinases (JAK)

Essential thrombocythemia, polycythemia vera, and primary myelofibrosis are usually part of a genetic mutation that occurs due to a protein known as Janus kinase 2 (JAK2). Because of this mutation, your cells can multiply rapidly without stopping.

Mutations Associated With The Calr Gene Or Mpl Gene

Individuals with primary myelofibrosis and essential thrombocythemia normally have CALR or MPL gene mutations.

Chromosome Errors

A chromosome is a part of a structure that has genes in it. Individuals with CML (Chronic myelogenous leukemia) have a particular error that involves their chromosomes. In a condition like CML, your one chromosome changes places with your other chromosome, leading to the formation of the “Philadelphia chromosome.”

Even if these discoveries are useful, you should know these don’t indicate the actual causes of genetic changes. But still, with this data, your healthcare provider will be able to provide you with the right diagnosis and treatment to deal with these genetic mutations.

Understanding The Risk Factors For Myeloproliferative Neoplasms?

Talking about the risk factors for MPN, the common ones include family history, sex, and age. All these factors can increase your risk of developing myeloproliferative neoplasms.

Age: MPN is known to affect many age groups. However, in most cases, this cancer is found in people that are in their 50s and 60s.

Sex: Polycythemia vera is a common MPN type that is more common in males than females. On the other hand, essential thrombocythemia is more common in females than males. So, besides these two cancers, there are other ones that happen equally in both genders.

Furthermore, other than age and sex, researchers have found connections between MPNs, certain toxins, and radiation exposure. A few studies have even mentioned that individuals that have come under exposure to high radiation levels and toxins such as benzene are at a higher risk of developing MPNs, like chronic myelogenous leukemia, polycythemia vera, and primary myelofibrosis.

How Is Myeloproliferative Neoplasm Diagnosed?

For people with MPN, your provider will learn about your medical and family history. In addition, the doctor will run a physical exam in order to confirm the symptoms of MPN. So, to diagnose, the healthcare provider will perform various tests on the bone marrow and blood. Here are the different methods your doctor may use to diagnose myeloproliferative neoplasms.

Complete Blood Count

Your healthcare provider will run this test to measure the levels of your blood cell. If you have essential thrombocythemia, the doctor will evaluate your platelet levels. On the other hand, if you have polycythemia vera, the provider will check for enhanced hemoglobin. This means confirming the amount of protein in your red blood cells. In addition, it includes checking platelets and white blood cells.

Bone Marrow Biopsy

The doctor may perform bone marrow aspiration, which is another name for bone marrow biopsy, to diagnose your condition. For this test, your provider will take your bone marrow sample to confirm the blood cells. After this, medical pathologists will examine your blood tissue and cells using a microscope to determine the differences between abnormal and normal cells.

The healthcare providers will try to confirm whether you contain an odd number of stem cells through this test. In addition, the providers will look for chromosomes changes 

and genetic mutation signs which will help confirm the MPN type you have.

Peripheral Blood Smear (PBS)

PBS helps display abnormal cell shapes indicating a disease. By running the blood chemistry tests, you can get details on the number of chemical types present in the blood, such as glucose, enzymes, protein, etc. Your doctor can know how well your organs are working through these numbers. And sometimes, these numbers may suggest that you have MPN.

Genetic Testing

In genetic testing, doctors may check the blood cells to find changes in your genes. By checking these gene changes, the healthcare provider can know whether they can affect the production of blood cells.

Also Read: Esophageal Cancer: First Signs, Causes, Survival Rate, And Outlook

What Are Myeloproliferative Neoplasms Treatments?

Allogeneic stem cell transplantation is one possible cure for myeloproliferative neoplasms. This is also used as one of the treatment methods for MPN. However, not everyone can go through stem cell transplantation. The only reason why this treatment is not suitable for everyone is that the process can be difficult to handle.

Still, there are various treatment methods for MPN that can help you manage the symptoms. These treatments also help reduce the blood cells amount, offering you relief. Understand that myeloproliferative neoplasms treatments can vary based on your type and present symptoms. However, all the treatments have one general aim, to lower the count of abnormal blood cells.

So, first, let’s look at the different treatments available for treating MPNS. After that, we will see what treatments are particularly used to manage the symptoms of various types of myeloproliferative neoplasms.

Chemotherapy

In this treatment, the providers offer drugs that help eliminate extra blood cells in your body. There are two ways to perform chemotherapy: take the pills orally, or your doctor will administer it to your body through an IV. This treatment is referred to as symmetric treatment because the drugs enter your blood treatment, move through your body, and kill cells throughout your body.

Biological Therapy

Your doctor may perform biological therapy as a possible treatment for your MPN. In this therapy, the healthcare provider may use materials made in a laboratory or by the body to restore, direct, or boost your immune system. This therapy is also known as immunotherapy or biological response modifier (BRM). 

Radiation Therapy

The healthcare provider uses high-energy X-rays in this therapy to eliminate abnormal blood cells and relieve symptoms. Your doctor may perform external beam radiation therapy, in which a machine will be used outside your body to direct the radiation. This radiation therapy type is often used to treat myeloproliferative neoplasms. Also, your doctor may offer you a radioactive drug known as P32 through an IV to lower abnormal red blood cells produced by your bone marrow.

Hormone Therapy

In some cases, your doctor may use hormone therapy to treat your MPN side effects. Patients with agnogenic myeloid metaplasia may get glucocorticoid hormones from their providers, which can help increase the longevity of red blood cells. Also, your doctor may give you androgens that will help stimulate the bone marrow so it can make more blood cells.

How you’ll respond to the therapy or how fast your recovery will depend on your MPN type, present age, as well as overall health. In most cases, people with polycythemia vera and essential thrombocythemia will live more than 10 to 15 years without facing too many complications. Furthermore, individuals having myelofibrosis typically live for five years. However, in rare cases, this condition may develop into acute leukemia.

Surgery

Spleen, as mentioned, is one of the common symptoms of MPN. So, your healthcare provider may perform a splenectomy to remove the spleen. Surgery is only done if your spleen is swollen, causing you too much discomfort.

Stem Cell Transplantation

As mentioned, allogeneic stem cell transplantation can potentially treat or cure myeloproliferative neoplasms. This treatment involves transferring stem cells from one individual to another.

Now, whether this treatment is suitable for you will depend on the following factors:

• Age
• Present symptoms
• MPN subtype
• Extent of disease
• Donor availability

However, consulting with a professional before taking a stem cell transplantation is essential.

So, now we know the possible treatments for myeloproliferative neoplasms. Let’s check which treatment is generally suitable for your type of MPN.

Chronic Eosinophilic Leukemia

As mentioned, your treatment for your condition will depend on your MPN type. In the case of chronic eosinophilic leukemia, the doctor may recommend chemotherapy, immunotherapy, or corticosteroids to lower eosinophil levels.

Chronic neutrophilic leukemia

The treatments may include stem cell transplants, chemotherapy, and immunotherapy.

Chronic myelogenous leukemia

Usually, targeted therapy is given for this condition to prevent cells from having abnormal growth rates. Other treatments for this cancer include radiation therapy, chemotherapy, stem cell transplants, and immunotherapy.

Polycythemia vera

The healthcare provider commonly uses phlebotomy for treating polycythemia vera. In this treatment, the doctor will continuously remove blood to lower the blood volume as well as draw out abnormal blood cells. But, if you face some symptoms, your healthcare provider will give you targeted therapy to prevent the abnormal multiplying of cells.

In addition, your doctor may suggest medications such as aspirin to lower blood clot risk. This may help manage the abnormal red blood cells.

Essential thrombocythemia

If you have no symptoms, your healthcare provider, instead of prescribing treatments, will closely monitor your condition. However, if symptoms appear, the doctor will suggest treatments that may help control the multiplying of red cells. Alternatively, your doctor may suggest some medicines to lower the blood clot risk and prevent the bone marrow from producing platelets in abnormal amounts.

Primary myelofibrosis

The doctor will stick to monitoring your condition if there are no symptoms appearing in you. However, if you start experiencing symptoms, your healthcare provider may suggest some drugs or treatments to help treat anemia. Taking one example, in case the bone marrow isn’t producing the required red blood cells, your doctor may suggest a blood transfusion for you.       

In addition, the provider may suggest some medicine that can help stimulate the bone marrow so it makes enough blood cells. Furthermore, other treatments can also help treat this condition, including chemotherapy, stem cell transplant, immunotherapy, targeted therapy, and radiation therapy.

Myeloproliferative Neoplasms Survival Rate

If you have MPN, you may question how long you can live with this condition. Well, the survival rate for myeloproliferative neoplasms depends on several factors. It mainly involves how early you learn about your cancer and how the treatment works. Due to this, the experiences of patients vary significantly.

If you get diagnosed and treated at the right time, your chances of living for several years will increase significantly. And this careful treatment and monitoring have helped many patients to live for many years. Presently, there isn’t any expected outcome or prognosis for MPNs. However, if we talk generally, individuals who are diagnosed with myeloproliferative neoplasms are alive after five years.

So, talking about the survival rates for particular MPNs, these are as follows:

Chronic Myelogenous Leukemia

Survival rates of patients with CML have increased significantly because of the better effectiveness of new targeted therapies. The five-year survival rate for chronic myelogenous leukemia is 90%.

Essential Thrombocythemia

In this condition, generally, most people live for several years. However, taking medicines that help prevent blood clots is crucial, as this condition can lower your lifespan if left untreated.

Chronic Neutrophilic Leukemia And Polycythemia Vera

Most individuals can live for over 20 years if they follow careful management after diagnosis.

Primary Myelofibrosis

Individuals live around 5 to 10 years after being diagnosed with primary myelofibrosis.

For whatever type of MPN you have, it is suggested that you ask your doctor about your prognosis. The healthcare provider can explain to you different factors that can affect the prognosis. Your doctor is aware of your condition as well as the risk factors of this condition, such as your general health and health. As all this can affect the prognosis, make you discuss it with your doctor.

Lastly, MPNs themselves are not fatal. However, some cancer types can make you prone to health conditions like stroke and heart attack.

Some Care Measure To Manage Myeloproliferative Neoplasms

Having MPN can be confusing sometimes because people generally don’t experience any symptoms. However, even if you don’t feel anything, it’s better to look for ways to keep the symptoms at bay. And if you’re going through treatment, you might look for methods to manage treatment symptoms and side effects.

Well, whatever your situation is currently, one thing is certain, you’ll need to live with this condition for several years. So, try following these care measures which may help you manage your condition better:

Knowing Your Health Condition

Firstly, it is vital that you know about your health situation completely because there are several MPN symptoms that don’t indicate severe conditions. However, there can be some symptoms that may show signs of severe medical conditions. Trying to guess which symptoms are more serious and which are less serious can be confusing.

Therefore, you should talk to your doctor about how this disease can affect you. Once you know what things you can experience in the future will make you more prepared to handle the situation.

Eating Healthy Meals

You can manage your condition more effectively if you eat healthy and balanced diets. You can consult a nutritionist if you’re unsure what foods to eat during this disease.

Managing The Stress

Stress is quite common when you have an illness like cancer. However, stressing too much can cause different complications in your body. Therefore, it is crucial that you consult your healthcare provider about the concerns which are bothering you. They’ll listen to you and will also provide you with some solutions that may make you worry less about your condition.

Doing Exercise

Performing exercise is one of the most effective ways that can help manage stress. Doing some physical activities and walking in nature may also uplift your mood.

Joining Support Groups

Myeloproliferative neoplasm is a rare disease; dealing with it alone can be stressful and tiring. Try joining some support groups, as they will have individuals who understand what you’re currently going through. Talking to them may give you relief and strength to fight your condition.

When Is The Right Time To Contact A Healthcare Provider?

As mentioned, you sometimes may not experience any symptoms while you have myeloproliferative neoplasms. So, if you start experiencing any of the MPN symptoms, contacting your doctor for a quick diagnosis is recommended.

MPNs are not life-threatening, but they can be fatal if left untreated for a long period. This rare condition usually starts in your bone marrow when it creates an abnormal amount of blood cells. Although this condition occurs due to genetic mutation, what triggers it is still unknown. During this time, it is vital that you look for the right help.

University Cancer Centers understand the importance of offering immediate care to cancer patients. Our team of medical professionals makes sure to learn about your family and medical history to give a proper diagnosis for your condition. We use different diagnosis methods to confirm the stage and the type of cancer you have. Then, we offer you a treatment plan through which you can treat or even cure your cancer successfully. To receive individualized care from our healthcare providers, contact us at (713)-474-1414 to schedule an appointment. Also, use our appointment form to share more details about your condition.