Mantle cell lymphoma (MCL) is a rare subtype (6%) of non-Hodgkin’s lymphoma, which is a cancer of the lymphatic system (immune system). The lymphatic system produces lymphocyte cells (B-cells and T-cells) that normally help protect the body against infection and disease and is composed of lymph nodes and blood vessels, the thymus, spleen, tonsils and adenoids, and some areas of the intestines. In mantle cell lymphoma, which grows slowly, the B-cell lymphocytes that are situated in an area of the lymph node known as the mantle zone develop abnormalities and become cancerous, causing enlarged lymph nodes, and eventually spreading widely throughout the lymphatic system and other areas of the body, such as the skin, small intestine, liver, bone marrow, and other organs. Mantle cell carcinoma mainly occurs in males over the age of 50. Mantle cell lymphoma is categorized by the following stages: Stage I – This stage of mantle cell lymphoma indicates early disease where the cancer is in only one lymph node region, one organ, or one area outside of the lymph node region. Stage II – This stage of mantle cell lymphoma represents locally advanced disease in which the cancer has spread (metastasized) to more than one lymph node region above or below the diaphragm or has spread to one organ or one area outside of the lymph node region. Stage III – In this stage of mantle cell lymphoma, the cancer has spread (metastasized) to lymph node regions both above and below the diaphragm, and possibly the spleen or an organ or other area of the body. Stage IV – In this stage of mantle cell lymphoma, the cancer has spread (metastasized) widely throughout one or more organs or areas of the body and may or may not involve lymph nodes. Mantle cell lymphoma is further described by either A (no systemic symptoms) or B (significant systemic symptoms, such as persistent fever, unexplained weight loss, and drenching night sweats). An additional category E indicates that the cancer has spread (metastasized) to one organ outside of the lymphatic system.

There are several alternative names for mantle cell lymphoma, including B-cell lymphoma, MCL, and Non-Hodgkin’s lymphoma (NHL).

While the causes of mantle cell lymphoma are unknown, researchers believe that lymphomas are formed when DNA abnormalities in lymphocyte cells occur due to a number of genetic and immunological abnormalities and environmental factors, such as aging, being a Caucasian male, having an immune disorder, such as autoimmune diseases, having certain genetic (chromosomal) disorders, being on immunosuppressants, and being infected with the human immunodeficiency virus (HIV), human T-cell leukemia virus, type I (HTLV-1), Epstein-Barr virus (EBV), or helicobacter pylori. Environmental risk factors for developing mantle cell lymphoma include exposure to ultraviolet rays, certain workplace chemicals, and ionizing radiation. Mantle cell lymphoma is characterized by an overproduction of a protein that stimulates cell growth (cyclin D1) caused by a rearrangement (translocation) between chromosomes 11 and 14.

While early-stage mantle cell lymphoma may not have any symptoms, the most common symptom is persistent, painless swollen lymph nodes around the neck and throat. Additional symptoms of mantle cell lymphoma may include enlargement of other lymph nodes, such as axillary (underarm), shoulder, elbow, chest, abdominal, and pelvic nodes, fatigue, paleness (pallor), headaches, bruising, loss of appetite, unexplained weight loss, nausea, vomiting, abdominal bloating or discomfort, enlargement of the liver (hepatomegaly) and/or spleen (splenomegaly) fever, intestinal polyps, drenching night sweats, anemia, confusion, personality changes, and seizures.

Treatments for mantle cell lymphoma depend on the stage of the disease and the patient’s overall health, but generally involve a combination of chemotherapy and immunotherapy drugs and radiation therapy. Stem cell transplant may also be used in some select patients. Chemotherapy – Chemotherapy uses drugs that kill cancer cells and is often combined with immunotherapy drugs for the treatment of mantle cell lymphoma. Chemotherapy may be administered orally (pill), intravenously (IV), or in severe cases, intrathecally (directly into the spinal fluid; methotrexate) and is given in cycles. Chemotherapy drugs for mantle cell lymphoma may also be given in combinations as drug regimens also in combination with corticosteroids, such as cyclophosphamide (Cytotoxan), vincristine (Oncovin), and prednisone (Deltasone), known as CVP, or cyclophosphamide, doxorubicin (Adriamycin), hydroxydaunorubicin (Rubex), vincristine (Oncovin), and prednisone (Deltasone), known as CHOP, as well as other combination regimens. Immunotherapy (Biological therapy) – Immunotherapy drugs use the patient’s immune system to attack cancer cells or to prevent them from growing and are often used for mantle cell lymphoma in combination with chemotherapy drugs, such as chemotherapy plus rituximab (Rituxan) or bortezomib (Velcade), for relapsed or untreated mantle cell lymphoma, or ibrutinib (Imbruvica), also for relapsed mantle cell lymphoma, or acalabrutinib (Calquence), for mantle cell lymphoma that has had at least one treatment, or lenolidomide (Revlimid), for relapsed or progressed mantle cell lymphoma after two prior therapies. Radiation therapy – Radiation therapy uses high-energy X-rays or protons to kill cancer cells and shrink tumors and may be administered for early or advanced stage mantle cell lymphoma. Stem cell transplant (Bone marrow transplant) – Different forms of stem cell transplantation may be used to treat mantle cell lymphoma in first complete remission. Stem cell transplantation uses bone marrow stem cells that are infused into the patient to help create new, healthy cells after using chemotherapy and irradiation to kill the unhealthy cells and may include for mantle cell carcinoma either autologous stem cell transplantation or reduced intensity allogenic stem cell transplantation, depending on the availability of a matched donor.

There are several online, local, national, and international support groups for mantle cell lymphoma, including the following: Leukemia and Lymphoma Society - https://www.lls.org/support/support-groups Lymphoma Support Network - https://lymphoma.org/learn/supportservices/lsn/ Smart Patients - https://www.smartpatients.com/forums/mantle-cell-lymphoma

While, in the past, mantle cell lymphoma had a poorer outcomes (prognosis), today the survival rate has doubled as most patients respond well to chemotherapy. For many patients, however, the disease may progress or return or treatment resistance to chemotherapy may develop which may lead to further treatments with different drugs or stem cell transplantation. The prognosis for the blastoid form of mantle cell lymphoma is poor, as this type usually progresses after chemotherapy.

Possible complications of mantle cell lymphoma mainly occur from progression of the disease and may include low white (cytopenia) and red blood cell counts (anemia), low platelets (cells responsible for blood clotting), high white blood cell counts (leukocytosis), gastrointestinal (polyps or obstruction), or central nervous system (CNS) complications. Cancer treatment-related complications may also occur, such as extreme fatigue, skin and nail changes, loss of hair, and increased susceptibility to infection, for which treatment is an essential part of cancer care.

If you experience any symptoms of mantle cell lymphoma, such as persistent fever, fatigue, or night sweats, and especially the appearance of a painless, swollen lymph node around the neck or throat, underarm, groin or other areas, make an appointment with your doctor as soon as possible.

While there is no known way to prevent mantle cell lymphoma, the risk of developing the disease may be reduced by avoiding infection with the human immunodeficiency virus (HIV) by practicing safe sex and avoiding illegal intravenous (IV) drug use.