Waldenstrom Macroglobulinemia - Symptoms and Diagnosis

Introduction

Waldenstrom macroglobulinemia (WM) is cancer that begins in the B cells. Cancer cells in WM patients resemble that of the cells involved in multiple myeloma and non-Hodgkin's lymphoma. Essentially, multiple myeloma is cancer involving the plasma cells, and non-Hodgkin's lymphoma is a cancer of lymphocytes (a type of white blood cells). WM cells have the characteristics of both plasma cells and lymphocytes and are called lymphoid traits.

WM cells produce large amounts of certain types of antibodies (immunoglobulin M or IgM) known as macroglobulins. All antibodies (proteins) produced by WM cells are the same, so they are called monoclonal proteins or simply M proteins. Accumulating this M protein in the body can cause many of the symptoms of WM, including excessive bleeding, blurred vision, and nervous system problems.

WM cells proliferate primarily in the bone marrow and can be populated with standard cells that make up different types of blood cells. This lowers the level of red blood cells (causing anemia) and can cause fatigue and weakness. It also reduces the white blood cell count, thereby impairing the body's ability to fight infections. The number of platelets in the blood will also decrease, increasing bleeding and bruising.

Lymphoma cells also grow in organs such as the liver and spleen, which can swell and cause abdominal pain.

What Causes Waldenstrom Macroglobulinemia?

• The cause of Waldenstrom macroglobulinemia is not clear.

• Doctors know that the disease begins with abnormal white blood cells and causes errors (mutations) in the genetic code. The error causes the cell to continue to increase.

• Cancer cells do not mature and die like normal cells, so they accumulate and ultimately overwhelm the production of healthy cells. Here the affected individual’s soft, blood-producing bone marrow tissue gets replaced by a cluster of Waldenstrom macroglobulinemia cells.

• Waldenström macroglobulinemia cells continue to try to make antibodies like healthy white blood cells but make abnormal proteins that the body cannot use. Eventually, this abnormal protein, which is of no use to the body, fills the blood and impairs blood flow, and causes complications.

• Waldenstrom macroglobulinemia is more common among the male population and usually diagnosed in those above the age of 65. One is at a higher risk of developing cancer if a family member has a history of Waldenström macroglobulinemia or another type of B-cell lymphoma.

What Could be the Risk Factors for Waldenstrom Macroglobulinemia?

Risk factors for Waldenstrom Macroglobulinemia includes:

• Age: The incidence is higher with advancing age. People above 50 years of age are at risk.

• Race: The disease is more prevalent in whites.

• Gender: Men are more prone when compared to women. But the reason is not known.

• Hereditary: It appears that inherited genes contribute to WM in at least some cases. A close relative with WM or a related B-cell disease, such as MGUS, specific forms of lymphoma, or leukemia, affects about 1 in 5 persons with WM.

• Hepatitis C: Chronic hepatitis C infection increases the risk for the disease.

• Autoimmune Diseases: Certain autoimmune diseases like Sjogrens syndrome, pose higher risk for the disease.

What Are the Symptoms Seen With Waldenstrom Macroglobulinemia?

Waldenstrom macroglobulinemia is slow-advancing. Hence it may not cause any signs or symptoms for many years.

When they occur, the following signs and symptoms may be seen:

• Bruises can form quickly.

• Bleeding from nose or gums.

• Fatigue.

• Weight loss.

• Numbness in the hands or feet.

• Fever.

• Headache.

• Shortness of breath.

• Changes in eyesight.

• Disorientation, dizziness, and clumsiness.

How Is Waldenstrom Macroglobulinemia Diagnosed?

The tests and procedures used to diagnose Waldenstrom macroglobulinemia are as follows:

• Blood Test: A blood test shows the number of healthy blood cells. Blood tests are also used to detect IgM proteins produced by cancer cells. Tests also measure organ function and reveal if the IgM protein affects organs such as the kidneys and liver.

• Bone Marrow Biopsy: In this process, the doctor collects a sample of the affected person’s hip bone marrow using a special type of needle. Samples are tested for cancer cells. If found, advanced laboratory analysis will help physicians understand the characteristics of cancer cells, including genetic mutations.

• Imaging Tests: Imaging tests help doctors determine if cancer has spread to other body parts. Imaging tests may include computer tomography (CT) scans or positron emission tomography (PET) scans.

How Is Waldenstrom Macroglobulinemia Treated?

Treatment options for Waldenstrom macroglobulinemia generally include

• Observation: If the IgM protein is found in the blood, but there are no signs or symptoms, one can wait for the start of treatment. The doctor may recommend a blood test every few months to monitor the condition. After that, they can go for years without further treatment.

• Plasma Exchange: If one notices signs or symptoms associated with too much IgM protein in the blood, it is recommended to undergo plasma exchange (plasma exchange) to remove the protein and replace it with healthy plasma.

• Chemotherapy:Chemotherapy drugs are those that kill rapidly growing cells, such as B. Waldenstrom's abnormal blood cells produced by macroglobulinemia. A single chemotherapy drug or a combination of drugs is administered as first-line treatment for people who develop signs or symptoms of Waldenström macroglobulinemia. In preparation for a bone marrow transplant, high-dose chemotherapy can also be used to suppress bone marrow production.

• Targeted Therapy: Targeted therapies kill cancer cells by targeting specific abnormalities that allow them to survive. Targeted therapies, alone or in combination with other medications such as chemotherapy or biotherapy, as first-line treatment for macroglobulinemia in Waldenstrom or when cancer recurs.

• Biological Therapy- Biological drugs use the immune system to kill cancer cells. Natural therapies combined with other drugs may be helpful for initial treatment or in treating recurrent Waldenström macroglobulinemia.

• Bone Marrow Transplant- In certain highly selected situations, bone marrow transplants, also known as stem cell transplants, can be used to treat Waldenström macroglobulinemia. During this procedure, high doses of chemotherapy are used to eradicate the affected bone marrow; healthy blood stem cells are injected into the body so that one can rebuild healthy bone marrow.

• Clinical Trials- Clinical trials offer the opportunity to try out the latest treatments for Waldenström macroglobulinemia.

Conclusion

Waldenstrom macroglobulinemia is a chronic slow-advancing lymphoproliferative disorder. It usually affects the elderly and is found primarily in the bone marrow, but it can also affect the lymph nodes and spleen. Affected people have high immunoglobulin M (IgM) antibodies, which can lead to blood thickening (hyperviscosity). Some people are initially asymptomatic and are diagnosed with regular blood tests, but common symptoms include weakness, loss of appetite, and weight loss. Other symptoms include peripheral neuropathy, fever, Raynaud's phenomenon, and changes in mental status. Blood hyperviscosity can cause nosebleeds, headaches, dizziness, blurred vision, or loss of sight. The underlying cause of the condition is still unknown, but environmental, genetic, and viral factors are suspected. There are several reports of familial cases suggesting a genetic predisposition.