Waldenstrom Macroglobulinemia (Lymphoplasmacytic Lymphoma): Introduction

Approved by the Cancer.Net Editorial Board, 03/2022

ON THIS PAGE: You will find some basic information about Waldenstrom macroglobulinemia and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to Waldenstrom Macroglobulinemia (Lymphoplasmacytic Lymphoma). Use the menu to see other pages. Think of that menu as a roadmap for this entire guide.

Waldenstrom macroglobulinemia is a type of non-Hodgkin lymphoma (NHL) that affects small lymphocytes, which are white blood cells.

About lymphoma and the lymph system

NHL is a term that refers to many types of cancer of the lymphatic system, which can have different symptoms and signs, physical findings, and treatment options.

The lymphatic system is made up of thin tubes that branch out to all parts of the body and helps fight infection. The lymphatic system carries lymph, a colorless fluid containing lymphocytes. Lymphocytes fight germs in the body. B-lymphocytes, also called B cells, make antibodies to fight bacteria. T-lymphocytes, or T cells, kill viruses and foreign cells and trigger the B cells to make antibodies.

Groups of bean-shaped organs called lymph nodes are located throughout the body at different sites in the lymphatic system. Lymph nodes are found in clusters in the abdomen, groin, pelvis, underarms, and neck. Other parts of the lymphatic system include the spleen, which makes lymphocytes and filters blood; the thymus, an organ under the breastbone; and the tonsils, located in the throat.

About lymphoplasmacytic lymphoma and Waldenstrom macroglobulinemia

Lymphoma begins when healthy cells in the lymphatic system change and grow out of control, which may form a tumor.

Lymphoplasmacytic lymphoma is an indolent or slow-growing form of B-cell NHL, and about 1% of people with NHL have this subtype. Lymphoplasmacytic lymphoma often involves the bone marrow, sometimes lymph nodes, and the spleen. In many people, this lymphoma produces a protein, called immunoglobulin M (IgM), that is found in the blood. If the lymphoma is producing IgM, the condition is called Waldenstrom macroglobulinemia. The majority of cases of lymphoplasmacytic lymphoma are Waldenstrom macroglobulinemia. This guide will use the term "Waldenstrom macroglobulinemia" to discuss both conditions.

Because lymphatic tissue is found in so many parts of the body, Waldenstrom macroglobulinemia can start almost anywhere and may spread to almost any organ in the body. When people are first diagnosed with the disease, it has usually already spread to the blood and bone marrow. It may also eventually affect the lymph nodes, liver, or spleen, and rarely the stomach, intestines, lungs, skin, or thyroid gland.

Because Waldenstrom macroglobulinemia is similar to some B-cell lymphomas or multiple myeloma, treatment can often be similar for these patients and you may be referred to see specialists for these diseases. You should talk with your health care team about your specific diagnosis and what treatment plan is best for you.

Learn more about the subtypes of NHL.

Looking for More of an Introduction?

If you would like more of an introduction, explore these related items. Please note that these links will take you to other sections on Cancer.Net:

  • ASCO Answers Fact Sheet: Read a 1-page fact sheet that offers an introduction to NHL. This free fact sheet is available as a PDF, so it is easy to print.

The next section in this guide is Statistics. It helps explain the number of people who are diagnosed with Waldenstrom macroglobulinemia and general survival rates. Use the menu to choose a different section to read in this guide.