Common Misconceptions About Waldenström Macroglobulinemia

Written by: Rob Levy
Medically Reviewed By: Jorge Castillo, MD

Waldenström macroglobulinemia, a rare form of non-Hodgkin lymphoma, has its share of eccentricities. 

One of the hallmarks of the disease, for example, is an overabundance of a particular protein, yet other conditions may also have that feature. It’s also not unusual for patients to have very high protein levels but have no symptoms or mild ones. And while most cancers are treated with the aim of eliminating them, treatment for Waldenström may have a different goal. 

We asked Jorge Castillo, MD, clinical director of Dana-Farber’s Bing Center for Waldenström macroglobulinemia, which provides comprehensive evaluation and treatment for patients with the disease, to discuss some common misconceptions about Waldenström and how they can be dispelled. 

Misconception 1: All people with elevated blood levels of the protein IgM have Waldenström. 

Fact: While increased IgM is a signature of Waldenström, a proportion of people with high protein levels have another condition, such as MGUS (a precursor of multiple myeloma), marginal zone lymphoma, or, rarely, IgM myeloma. 

Because each type of lymphoma and myeloma has a specific treatment, it’s critical that the diagnosis be accurate. When an individual is found to have high IgM, the next step should be a bone marrow biopsy, in which a pathologist experienced in diagnosing Waldenström examines a bone marrow sample looking for the characteristic malignant cells and genetic mutations linked to the disease. 

Misconception 2: Patients with Waldenström need to be treated immediately after they’ve been diagnosed with the disease. 

Fact: Treatment for Waldenström should begin when two criteria are met, Castillo says: 1) The patient’s symptoms are interfering with activities of daily life; and 2) it’s clear that the symptoms are arising from Waldenström and not some other condition. 

Understandably, patients with high IgM but no symptoms may be anxious to start treatment quickly, Castillo notes. But as long as their blood cell counts are normal, symptoms are absent, and there’s no hyperviscosity – thickening of the blood – it’s often advisable to monitor them rather than treat them right away. Research shows that patients who are asymptomatic have a normal life expectancy, so treatment is unlikely to help them live longer. Moreover, Waldenström therapies can produce side effects that lower patients’ quality of life. 

For patients with mild symptoms such as slightly swollen lymph nodes or low-level anemia, monitoring their condition can also be a sound approach, Castillo adds. When Waldenström does progress, it generally happens slowly, giving doctors and patients ample time to decide when to begin treatment. 

Misconception 3: The goal of treating Waldenström is always to achieve the deepest response possible – the biggest reduction in the amount of cancer. 

Fact: While that may be the best approach in some cases, it often makes sense to focus more on patients’ quality of life, Castillo comments. Some patients may benefit from treatments that produce only a superficial response – a small decrease in their IgM – but alleviate their symptoms for an extended period. In these cases, it’s especially important to give symptom-relieving therapies enough time to work. 

“As is true with many types of cancer, patients can become resistant to treatments used for Waldenström,” Castillo says. “For that reason, we don’t want to begin treatment too early or treat more extensively than necessary. Ideally, the goal is to use a treatment when it can truly be effective, so we can have other treatments in reserve if resistance develops.”