And according to a report by the American Cancer Society, the number of people diagnosed with this cancer is in the range of 1000-1500. Also, it is more common in older people. Since this cancer is very rare, many people are not even aware of its existence. And this is what we aim to help our readers with. That is why we are going to cover every aspect of this cancer and will also look at the available treatment options, discussing the life ahead for the ones who suffer from it, etc. 

NOTE: For the convenience of reading, we will be referring to Waldenstrom Globulinemia as WM. 

What Is Waldenstrom Macroglobulinemia? Is It The Same As Non-Hodgkin Lymphoma?

As we already saw, Waldenstrom Macroglobulinemia is a rare cancer that originates from the WBC and leads to the production of numerous abnormal WBCs. And when these cells accumulate in the blood, they start causing problems and complications. One common issue is irregularity in blood circulation.  

This cancer is also a type of non-Hodgkin’s lymphoma (NHL), which starts in the body’s lymphatic system and affects the ability to fight germs and diseases. 

The term “Waldenstrom” came after Jan G. Waldenstrom, the first person who described the condition in 1944. And the term “macroglobulinemia” refers to the excessive production of a large protein (immunoglobulin). 

Some other names for the condition are: 

• Macroglobulinemia of Waldenstrom
• Waldenstrom’s Macroglobulinemia
• Lymphoplasmacytic Lymphoma

Causes

The causes of WM are still unclear; however, a combination of changes in genetics is likely to be one. MYD88 and CXCR4 are the genes involved in signals that are sent within. And the variants within these genes cause the production of overactive (constantly turned on) proteins. 

When the signaling between these proteins occurs, it allows the abnormal WBC to survive. It prevents them from undergoing apoptosis (the process leading to the death of unwanted cells). And thus, the accumulation of abnormal cells carries on, which likely leads to WM cancer. 

How Rare Is Waldenstrom Macroglobulinemia? 

We also talked about the rarity of this cancer. However, the question is just how rare it is. Let’s find out!

WM cancer is so rare that just 3 out of a million are affected in a year in the United States. And around 1,000-1,500 people are diagnosed with this condition every year. While the origins of this cancer remain unknown, it is found to be more common in White Americans than Afro-Americans. That is what led to the belief in the role of genetic changes in the development of WM. Also, the condition is 2x more common in males and people in the 70s.

How Does WM Affect The Body?

Bone marrow is the center of bones which is spongy in nature. It is also responsible for producing stem cells that eventually turn into RBCs, WBCs, and platelets. 

However, that is also where the abnormal cells start developing. It is the normal B-cells (cells that regulate the immune system) in the body that turn into abnormal cells. The abnormality stems from the mutation or changes in the genes that manage the activity of B-cells. And when the B-cell turns into an abnormal cell, it starts reproducing similar cells. 

Then, the abnormal cells start producing IgM (immunoglobulin M), which is an antibody protein. It leads to the thickening of the blood, which is the hyperviscosity syndrome unique to WM. 

Since the flow of blood becomes difficult, it can cause 

• Tear in the blood vessels
• Bleeding from the nose, gums, or retinas in the eye.

Normally, the B-cells move into the bloodstream as they mature. And they eventually die there and get replaced with healthy B-cells. But, the production of abnormal cells in the bone marrow messes up that system. 

The reason for that is that, in WM, the abnormal cells keep on multiplying instead of dying like B-cells. It leads to crowding in the bone marrow, which hinders the development of normal blood cells. The situation can lead to: 

• Development of anemia
• Being more prone to infections
• It becomes easy to get cuts or bruises

Waldenstrom Macroglobulinemia Symptoms & Risk Factors

In some cases, people learn about the condition only after visiting a healthcare provider. So, it is not necessary for a person to have WM symptoms and can develop the condition for other reasons. Also, its symptoms come at a very gradual pacing for the ones who do experience them. 

The most common symptoms include: 

• Fatigue
• Fever
• Night sweats
• Weight loss
• Loss of appetite
• Blurry vision or loss of vision
• State of confusion 
• Getting cuts or bruises easily
• Tingling sensation in fingers and toes
• Nosebleeds
• Headache
• Short breath
• Swelling in the lymph nodes
• Spleen or enlarged liver

And even though the cause of the condition is unclear, there still are some risk factors associated with it. Let’s have a look at who is at risk: 

• People over 70 years old
• People with white ethnicity
• Males or those who are born males
• Someone with AIDS
• Those who have other family members suffering from WM or have a family history. 
• People who have been exposed to certain chemicals

What Are The Different Stages Of Waldenstrom Macroglobulinemia?

The determination of the cancer’s stage is very important. It helps in describing the extent of cancer in the body and the best way to proceed. Normally, cancer is divided into different stages to determine its severity. 

However, there is no standard of staging in the case of WM. The reason for it is that the extent of the disease is shown to be irrelevant to the outcome or the treatment option. And instead, the factors such as age, blood cell count, etc., seem more relevant from a medical perspective. These factors help in determining whether the person is at: 

• Low risk
• Intermediate risk
• High risk

While there is no staging system, there is certainly a system for prognosis that makes use of certain factors. We will cover that in detail in the later section of the blog. 

How Do You Diagnose Waldenstrom’s?

Waldenstrom’s or WM is mostly discovered when the person visits the doctor, which we already talked about. 

The doctors examine the person, and if they find any sign or symptom of WM, they proceed further with the diagnosis to ascertain. There are several tests that a doctor would prescribe to a WM patient. Apart from the tests, knowing the family history in regard to the condition and medical history also helps. 

If the doctor believes that the person may have the symptoms of WM, then further tests are recommended. These tests are as follows. 

Blood Tests

Since the cancer originates in the bone marrow and leads to irregular blood flow, blood tests can become extremely helpful in identifying the disease. They identify the production of IgM protein that the abnormal cells produce. 

Also, blood tests can help in identifying the organs which may be affected by the abnormal cells. It can affect the kidneys as well as the liver.

Also Read: What Causes Soft Tissue Sarcoma? Can It Be Cured? 

Bone Marrow Sample Test  

In a bone marrow biopsy, the doctor uses a needle to extract a sample from the bone marrow in the hipbone. The sample is then used for detecting the presence of cancer cells. And if there is the presence of the cells, then the doctor would proceed to a more advanced analysis to get a better understanding of the cancer cells’ characteristics. 

Imaging Tests

There are many imaging tests that can help in determining the spread of cancer. These tests include 

• CT scan
• PET scan
• X-ray
• MRI scan
• Ultrasound

Viscosity

The test of viscosity measures the thickness of blood in the system. That is because the abnormal cells make the blood thicker, making its flow to be irregular. It also gives a brief idea about the IgM protein level in the blood. 

Is There A Cure For Waldenstrom Macroglobulinemia? (Treatment Options)

As of now, there is nothing that can cure WM cancer completely. And one possible reason for this may be the rarity that leads to a lack of research. Even though there is no cure, there certainly are treatment options available. 

These treatment options may even help in prolonging the life of those individuals who suffer from it. The treatment can bring the symptoms under control (to some extent). They are as follows: 

• Plasma Exchange
• Chemotherapy
• Targeted Therapy
• Biological Therapy
• Bone Marrow Transplant

According to many healthcare experts, the treatment for WM should start only when the disease starts becoming problematic. It is because there are side effects of many of those treatments. There are also many studies that indicate that delaying treatment in this manner does not affect the survival rate much. 

Also, understand that every treatment works differently and is not suitable for everyone. Various factors, such as age, symptoms, medical history, etc., come in for the choice of the right treatment option. 

Is There A New Treatment For Waldenstrom Macroglobulinemia?

As the research for this cancer progresses, so do the treatment options for WM. New treatments for Waldenstrom Macroglobulinemia keep popping up. And it is quite obvious that some of them will be more effective than others. 

Over the years, targeted therapy has come out as an effective treatment option overall. There are several targeted therapies, some of which come under kinase inhibitor therapy. 

Is It Possible To Survive 20 Years With Waldenstrom Macroglobulinemia? (Prognosis)

Before we answer the question of whether it is possible to survive 20 years with WM, let’s understand the survival and prognosis of this cancer. 

The term that is common in the field of cancer when it comes to cancer is the 5-year survival rate. If you are not familiar, then it is essentially the calculation of survival percentage for 5 or more years after the diagnosis. While the survival rate may not tell the whole story, it does give an estimate of the survival chance.  

Before we look at the numbers, let’s have a look at the factors that determine the prognosis of WM. The system has been developed by ISSWM (International Prognostic Scoring System for Waldenstrom Macroglobulinemia). The factors are: 

• Age
• Blood hemoglobin level
• Monoclonal IgM level
• Platelet count
• Beta-2 microglobulin level 

Using these factors, a person can be put into the risk groups which we already talked about. Now, let’s look at the 5-year survival for the different risk groups. 

The overall 5-year survival for WM is 78%. You also need to understand that these numbers do not translate to an individual’s survival. They only tell what there is already. 

Now, coming back to the question. There have also been reports of patients diagnosed with WM to have survived for 10-12 years. However, there has not been any reported case of someone surviving for 20 years. And one of the most plausible reasons for that is that WM is most common in older individuals. Given the physical capabilities of old people and the strenuous treatments, survival for 20 years seems very difficult. 

What Are The Best Dietary & Lifestyle Habits For Waldenstrom Macroglobulinemia?

There are various dietary and lifestyle habits that may help in preventing WM. These habits may also help in prolonging the life of WM patients. Some healthier habits include: 

• Washing hands properly 
• Avoid contact with people who may be infected with flu or cold. 
• Exercise regularly to keep the flow of blood properly maintained. 
• While there is no special diet to prevent or treat WM, it is always a good idea to have a balanced diet. Eat more fruits and vegetables, which are good for your overall health. Also, have whole grains, foods with low fat, and red meat. 
• Make sure to get proper sleep to relieve some of your stress. That is because stress and depression are very common among cancer patients. 

Conclusion

Waldenstom’s Macroglobulinemia is a very rare cancer that can also be life-threatening. And the best way to avoid developing this or any other cancer is to incorporate healthy habits into your daily life. While it may not guarantee prevention, it will certainly help in fighting against it.  

Also, because WM is so rare, consult only a doctor or an institution that specializes in cancer diagnosis and treatment. You can also reach out to University Cancer Centers, which is an institution that specializes in providing diagnosis and treatment plans to cancer patients. Our team will make sure to answer your every query and guide you in the direction that’s best for the patient.

Frequently Asked Questions