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Cutaneous B-Cell Lymphoma (CBCL)

Overview
Types of Cutaneous B-Cell Lymphoma
Treatment Options
Treatments Under Investigation
Clinical Trials
Follow Up
Support
Resources

Overview

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Lymphoma is the most common blood cancer. The two main forms of lymphoma are Hodgkin lymphoma and non-Hodgkin lymphoma (NHL). Lymphoma occurs when cells of the immune system called lymphocytes, a type of white blood cell, grow and multiply uncontrollably. Cancerous lymphocytes can travel to many parts of the body, including the lymph nodes, spleen, bone marrow, blood, or other organs, and form a mass called a tumor. The body has two main types of lymphocytes that can develop into lymphomas: B-lymphocytes (B-cells) and T-lymphocytes (T-cells)

B-cell lymphomas account for 85 percent of all NHL cases in the United States. The disease affects men and women equally, and can affect any age group. There are many different forms of B-cell lymphoma.

Lymphomas that arise in organs or tissues outside of the lymphatic system are called extranodal lymphomas. When extranodal lymphomas originate in the skin (making up 18 percent of all extranodal lymphomas and five percent of all NHLs), and there is no evidence of disease outside of the skin, they are called primary cutaneous lymphomas.Primary cutaneous B-cell lymphomas (CBCLs) occur when the lymphoma cells originate in B-cells.

CBCLs are nearly always indolent (slow-growing). They may appear on the skin as a reddish rash, lump, or nodule and may have a slightly raised and smooth appearance. The disease tends to recur on the skin, but will rarely develop into a systemic (throughout the body) lymphoma. Prognosis is usually very good, although it can commonly relapse in skin, with nearly 50 percent of those diagnosed with CBCL experiencing recurrence after an initial complete response to treatment.

Types of Cutaneous B-Cell Lymphoma

Primary Cutaneous Follicle Center Lymphoma is the most common type of CBCL. These skin lymphomas develop slowly over months or years. They usually appear on the head, neck, or trunk of the body as single or multiple tumors or nodules with a pink or reddish appearance.

Primary Cutaneous Marginal Zone B-cell Lymphoma is the second most common form of CBCL. This slow-growing lymphoma appears as pink or red lesions, nodules, and/or tumors, most commonly found on the extremities, although they can occur anywhere on the body.

Primary Cutaneous Diffuse Large B-cell Lymphoma, Leg-type is less common than other CBCLs, but is usually more aggressive (fast-growing), developing over weeks or months. This lymphoma usually appears as red or bluish-red lesions on the lower legs, although lesions can occur on any part of the body. The lesions frequently grow into large tumors that extend deep into the body. The lesions may become open sores and spread outside the skin more frequently than the slow-growing CBCLs.

Primary Cutaneous Diffuse Large B-cell Lymphoma, Other describes a group of very rare lymphomas, including intravascular large B-cell lymphoma, T-cell rich large B-cell lymphoma, plasmablastic lymphoma, and anaplastic B-cell lymphoma. Although these lymphomas usually appear on the head, trunk, and extremities, they are almost always systemic lymphomas with cutaneous symptoms and thus do not truly fit among the CBCLs.

Treatment Options

Treatment selection for CBCL depends on symptoms, general health, and stage of disease. Depending on the number of lesions present, primary cutaneous follicle center lymphoma and cutaneous marginal zone lymphoma may be observed or treated locally with either radiation or surgery. Secondary therapy for relapsed CBCL that recurs following initial remission can include observation, surgery, topical treatments, injected steroids, or radiation. Slow- growing CBCLs rarely transform into more aggressive types of lymphoma. For diseases that become widespread or systemic, a variety of chemotherapy regimens may be used. In these cases, therapies such as rituximab (Rituxan), either alone or in combination with chemotherapy, are often used.

There is no known best treatment strategy for the more aggressive types of CBCL, such as cutaneous diffuse large B-cell lymphoma, leg type. For single tumors, radiation is often effective, as are combinations of chemotherapy, or radiation and chemotherapy given sequentially. Like other aggressive forms, R-CHOP (rituximab, cyclophosphamide, doxorubicin hydrochloride, vincristine sulfate, prednisone) plus radiation therapy is commonly used to treat this type of B-cell lymphoma. Clinical trials are also often recommended. All of these treatments usually result in disease remission; however, relapse is common.

Treatments Under Investigation

Many treatments at various stages of drug development are currently being tested in clinical trials and for relapsed or refractory CBCL, including panobinostat, lenalidomide, bendamustine, and others. It is critical to remember that today’s scientific research is continuously evolving. Treatment options may change as new treatments are discovered and current treatments are improved. Therefore, it is important that patients check with LRF or with their physician for any treatment updates that may have recently emerged.

Clinical Trials

Clinical trials are crucial in identifying effective drugs and determining optimal doses for lymphoma patients. Patients interested in participating in a clinical trial should talk to their physician or contact LRF's Helpline for an individualized clinical trial search by calling (800) 500-9976 or emailing helpline@lymphoma.org.

Follow Up

Patients in remission should have regular visits with a physician who is familiar with their medical history as well as with the treatments they have received. Survivors and their caregivers are encouraged to keep copies of all medical records and test results as well as information on the types, amounts, and duration of all treatments received. This documentation will be important for keeping track of any effects resulting from treatment or potential disease recurrences. For further information, please review our fact sheet on survivorship issues.

Support

A lymphoma diagnosis often triggers a range of feelings and raises many concerns. In addition, cancer treatment can cause physical discomfort. Support groups and online message boards can help patients connect with other people who have lymphoma. One-to-one peer support programs, such as LRF's Lymphoma Support Network, match lymphoma survivors (or caregivers) with volunteers who have gone through similar experiences.

Lymphoma survivors should receive regular medical exams from a physician who is familiar with their medical history as well as the treatments they have received. Survivors and their caregivers are encouraged to keep copies of all medical records and test results as well as information on the types, amounts and duration of all treatments received. This documentation will be important for keeping track of any effects resulting from treatment or potential disease recurrences.

Resources

LRF offers a wide range of resources that address treatment options, the latest research advances, and ways to cope with all aspects of lymphoma. LRF also provides many educational activities, from in-person meetings to teleconferences and webcasts. For more information about any of these resources, visit the website at www.lymphoma.org or contact the Helpline at (800) 500-9976 or helpline@lymphoma.org.

The Cutaneous Lymphoma Foundation is an independent nonprofit patient advocacy organization dedicated to supporting every person with cutaneous lymphoma by promoting awareness and education, advancing patient care, and facilitating research. To find out more information about the resources that the Cutaneous Lymphoma Foundation provides, please visit their website (www.clfoundation.org).

Webcasts/Podcasts
Teleconferences
In-person Educational Programs
Support Programs

View or order the following publications from our booklets/factsheets webpage:
• Cutaneous B-Cell Lymphoma Fact Sheet

Understanding Non-Hodgkin Lymphoma: A Guide for Patients, Survivors and Loved Ones