This is the eleventh part of our series about the condition based on our patient booklet “Fast Facts for Patients: Waldenström Macroglobulinemia”. This article shows what to do when Waldenström macroglobulinemia (WM) comes back. Furthermore, approaches in research as well as new treatments are addressed.

When WM Comes Back

When WM comes back, it can be treated again. The type of treatment will depend on the effects of previous treatments, how long it is since the last course of treatment and your general health. The same treatment can be used again if a year or more has passed since its initial use.

If the WM relapses more quickly than this, a different drug or a combination of drugs or a stem cell transplant might be considered.

In most people with WM, relapsed disease responds well to treatment and further remissions can be expected.

High-Grade Transformation

In a small number of people, WM turns into a faster-growing type of lymphoma. If this happens, it usually causes new symptoms and is detected by tests such as a lymph node biopsy. This is called transformation and, although it sounds worrying, it can be treated using more intensive chemotherapy that is normally used for high-grade lymphomas.

Active Monitoring

It is very important that all treatment decisions are made with good judgment and care. A new treatment should be started for clinical reasons (symptoms) and not at the first sign that the IgM paraprotein level is rising.

The time it takes for a relapse to result in symptoms can span many months or even a few years. So, in the absence of emergency situations such as hyperviscosity, you may have a period of active monitoring that lasts a few months before treatment is started.

Research and New Treatments

Clinical Trials

You may be asked if you would like to take part in a clinical trial. Clinical trials are research studies that test new medical treatments or different combinations of existing treatments.

Some newer treatments are only available in a clinical trial. Not all hospitals take part in clinical trials and there may not be a trial that is suitable for you when you are diagnosed or relapse, but this is something to discuss with your specialist when planning treatment.

You do not have to take part in a clinical trial – you can always opt to have the standard treatment instead.

Studying Genetic Changes

Research is looking at the significance of genetic mutations such as MYD88 and CXCR4 mutations to see if medications can target them to disrupt the disease. Other genetic changes have been identified and are also being studied.

CAR T-Cell Therapy

You may have heard about a new type of treatment called CAR T-cell therapy (CAR stands for chimeric antigen receptor). It is currently being developed to treat different types of B-cell leukemia and lymphoma, but it is not yet a viable option for patients with WM.

Registry Data

Apart from clinical trials of new therapies, efforts are under way to capture so-called “real-world data” using registries, which are comprehensive electronic databases with data protection measures in place. In a rare condition like WM, this kind of evidence can help researchers understand how people live with their WM, and the effects of different treatments on their WM and their lives. This information can also help healthcare funders decide on the value of treatments in the care of patients.

Check out the other posts of our series here:

• What Is Waldenström Macroglobulinemia?
• What Causes Waldenström Macroglobulinemia and How Will It Affect Me?
• Waldenström Macroglobulinemia: Possible Effects
• Waldenström Macroglobulinemia: Who Is in My Care Team and Which Tests Will I Need to Have?
• Waldenström Macroglobulinemia: Common Feelings When Diagnosed and How You Can Help Yourself
• Waldenström Macroglobulinemia: Active Monitoring, Starting Treatment, and Follow-Up
• Waldenström Macroglobulinemia: Types of Treatment – Chemotherapy
• Waldenström Macroglobulinemia: Further Types of Treatment (Part 1)
• Waldenström Macroglobulinemia: Further Types of Treatment (Part 2)
• Waldenström Macroglobulinemia: What Are Supportive Treatments?

Information based on Fast Facts for Patients: Waldenström Macroglobulinemia (Karger, 2022).