Waldenström's disease, also known as Waldenström's macroglobulinemia or lymphoplasmacytic lymphoma, is a rare form of blood cancer. In this disease, the body produces too many abnormal white blood cells that closely resemble plasma cells. These cells mainly settle in the bone marrow, but can also spread to the lymph nodes, liver, and spleen.

What makes Waldenström's disease distinctive is that the abnormal cells produce large amounts of a protein called IgM, also known as M-protein or macroglobulin. Normally, this protein is only present in small amounts as part of the immune system. In Waldenström's disease, this balance is disrupted, leading to a range of physical symptoms.

The disease belongs to the group of non-Hodgkin lymphomas and is classified as an indolent, or low-grade, lymphoma. This means it usually behaves non-aggressively and tends to develop slowly.

How Common Is It?

Waldenström macroglobulinemia is a rare disease. Each year, several hundred people in the Netherlands are diagnosed with it. It occurs more often in men than in women and is usually diagnosed later in life, with the average age at diagnosis ranging from 60 to 70 years. The exact cause of the disease remains unknown.

Symptoms

Not everyone with Waldenström's disease experiences symptoms. In some cases, the disease is discovered by chance through blood tests, before any symptoms appear. When symptoms do occur, they often resemble those of other non-Hodgkin lymphomas:

Common general symptoms include weight loss, reduced appetite, fever, and night sweats. Lymph nodes may become swollen, and the spleen or liver may enlarge.

Because the production of healthy blood cells is disrupted, symptoms such as fatigue from anemia, an increased tendency to bleed, and a higher susceptibility to infections are also common.

A specific feature of Waldenström's disease is hyperviscosity: the high level of abnormal IgM protein in the blood makes it thicker than normal. This can cause blurred vision, headaches, dizziness, shortness of breath, and extra strain on the heart.

In about one in five people, peripheral neuropathy also develops, damage to the peripheral nerves. This can cause tingling, numbness, a "pins and needles" sensation, or muscle weakness.

Diagnosis

Diagnosis is usually made through blood tests, which reveal an elevated IgM level. Additional testing may include a bone marrow biopsy, ultrasound, a CT scan, or a PET-CT scan, depending on the individual situation.

Treatment and Outlook

Waldenström's disease cannot currently be cured, but it can be treated effectively. Because the disease usually grows slowly, treatment isn't always started right away. Doctors often choose a watch-and-wait approach as long as there are few or no symptoms.

If symptoms worsen, several treatment options are available, including chemo-immunotherapy, targeted therapy, and, in some cases, stem cell transplantation. In cases of acute hyperviscosity, plasmapheresis may be needed, a dialysis-like procedure that filters the excess protein out of the blood.

Life expectancy varies significantly from person to person and depends on factors such as age, blood values, and disease progression. On average, life expectancy after diagnosis is around 7 to 10 years, but people without symptoms can live with the disease for decades.