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NCCN Guidelines for Patients®: Mantle Cell Lymphoma, 2021 3 NCCN Guidelines for Patients®: Mantle Cell Lymphoma, 2021 4 Mantle Cell Lymphoma Contents 6 MCL basics 11 Testing 21 Treatment options 29 Treatment for advanced MCL 37 Early MCL and slow MCL 40 Making treatment decisions 49 Words to know 52 NCCN Contributors 53 NCCN Cancer Centers 54 Index NCCN Guidelines for Patients®: Mantle Cell Lymphoma, 2021 5 1 MCL basics 7 What is mantle cell lymphoma? 9 How common is mantle cell 7 What is the lymph system? lymphoma? 8 What is a mantle cell? 10 Can mantle cell lymphoma be cured? 8 What causes mantle cell lymphoma? 10 Review 9 How is mantle cell lymphoma identified? NCCN Guidelines for Patients®: Mantle Cell Lymphoma, 2021 6 1 MCL basics What is mantle cell lymphoma? Mantle cell lymphoma is a rare but What is the lymph system? treatable cancer of blood cells in lymph nodes and the lymph system. It’s often a The lymph system is part of the immune fast-growing cancer, but new treatments (infection-fighting) system. The lymph system every year are giving people more hope transports fluids to the bloodstream and fights and more years to live. germs. It’s made up of lymph, lymph nodes, and other organs. Lymph is a fluid that contains germ-fighting What is mantle cell lymphoma? lymphocytes. Lymph nodes (sometimes called “glands”) are small, bean-shaped structures. Hundreds of lymph nodes are located Mantle cell lymphoma (MCL) is cancer that throughout your body. As lymph fluid travels occurs in certain white blood cells. These through the body, lymph nodes catch and filter white blood cells are called lymphocytes. They out foreign particles and harmful cells. Lymph protect the body from infection. An abnormal nodes are usually clustered in groups in your growth of these lymphocytes is called a neck, chest, armpits, groin, pelvis, and along lymphoma. A lymphoma commonly affects the your gut. The spleen, tonsils, and thymus are lymph system, but it can also affect other parts also part of the lymph system. of the body. Lymph nodes There are hundreds of small bean-shaped structures throughout the human body called lymph nodes. Groups of lymph nodes are clustered in your neck, chest, armpits, groin, pelvis, and along your gut. Lymph nodes catch and filter out foreign particles and harmful cells, including cancer cells. NCCN Guidelines for Patients®: Mantle Cell Lymphoma, 2021 7 1 MCL basics What is a mantle cell? When you get an infection, an army of lymphocytes fills up your lymph nodes to fight the germs. That’s why the lymph nodes in your neck feel swollen when you have a cold, flu, or sinus infection. What is a mantle cell? What is cancer? Inside the lymph node are little round clumps called follicles. These follicles contain mostly B cells, a type of lymphocyte that plays a Cancer is a disease where cells—the key role in the immune system. Surrounding building blocks of the body—grow out of the center of each follicle is a mantle zone. control. This can end up harming the body. Imagine that the center of the follicle is a planet, like Saturn. The mantle zone is like the There are many types of cells in the body, ring that surrounds the planet. Mantle cells are so there are many types of cancers. B cells that lie in this mantle zone. Cancer cells don’t behave like normal cells. Cancer cells develop genetic errors that cause them to make many more cancer What causes mantle cell cells. The cancer cells crowd out and lymphoma? overpower normal cells. Cancer cells avoid normal cell death. They Researchers aren’t sure what actually causes also can spread far through blood or lymph this type of lymphoma. Most people who to other areas of the body. They can replace are diagnosed with MCL have developed many normal cells and cause organs to an unusual trait. This unique trait leads to a stop working. change in the B lymphocytes in the mantle zone in the lymph system. This change Scientists have learned a great deal about produces a buildup of a protein called cyclin cancer. As a result, today’s treatments work D1. This protein stimulates these abnormal much better than treatments in the past. cells to grow out of control and become Also, many people with cancer have more lymphoma cells. than one treatment choice. The big question is: What causes this unusual trait to occur in some B lymphocytes? Researchers don’t have an answer to that yet, but they’re working on it. NCCN Guidelines for Patients®: Mantle Cell Lymphoma, 2021 8 1 MCL basics How is mantle cell lymphoma identified? How is mantle cell lymphoma It’s important to identify your specific type of identified? lymphoma to make sure you get the right care and treatment. MCL is often discovered when a person has painless, swollen lymph nodes in the neck, armpit, or groin.