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Cutaneous B Cell Lymphoma (CBCL)

Cutaneous B Cell Lymphoma (CBCL) is a type of lymphoma that is almost always slow growing, and it has a few different subtypes. 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 go away and then return on the skin, but it rarely develops into a disease that affects other areas of the body. Nearly 50 percent of patients diagnosed with CBCL experience a recurrence after an initial complete response to treatment. Prognosis is usually very good.

What are the Common Symptoms

Common symptoms include a rash or nodules over any part of the body which are different depending on the type of lymphoma, itch and fatigue.

Types of Cutaneous B-Cell Lymphoma

Primary Cutaneous Follicle Centre 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 torso of the body as a red-brown pimply rash or nodules.

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 tumours, most commonly found on the torso or arms.

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 solitary or multiple tumour nodules on the legs and/or torso. The lesions frequently grow into large tumours that extend deep into the body. The lesions frequently grow into large tumours 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. These lymphomas usually appear on the head, torso, and extremities.

How is it Treated

Treatment selection for CBCL depends on the type of CBCL, the patient’s symptoms and overall health, and the stage of the disease. Depending on the number of lesions present, primary cutaneous follicle centre lymphoma and cutaneous marginal zone B-cell lymphoma may be observed (a method known as “active surveillance” also known as “watchful waiting”) or treated locally with either radiation therapy or surgery. Secondary therapy for relapsed (disease returns after treatment) CBCL 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 (systemic), a variety of 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 tumours, radiation is often effective, as are combinations of chemotherapy, or radiation and chemotherapy given sequentially. Similar to other B-cell NHLs, R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) plus radiation therapy is commonly used to treat CBCL that has spread and become a more generalized lymphoma. Clinical trials are also often recommended. All of these treatments usually result in disease remission, but relapse can happen

Treatments Under Investigation

Many treatments at various stages of drug development are currently being tested in clinical trials for relapsed or refractory (disease does not respond to treatment) CBCL, including panobinostat (Farydak), lenalidomide (Revlimid), bendamustine (Treanda), 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.