.jpg)
B-cell lymphomas account for the majority of nodal lymphomas, whereas primary cutaneous B-cell lymphomas (CBCLs) represent 20-25% of all cutaneous lymphomas. Because CBCLs have an overall favorable prognosis, proper recognition is vital for appropriate therapy and to avoid overtreatment in most cases. The tumor type and the extent of cutaneous involvement are the 2 most relevant prognostic factors in primary CBCL.
Types of Cutaneous B-Cell Lymphoma
Primary Cutaneous Diffuse Large B-cell Lymphoma 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.
Intravascular Large B-Cell Lymphoma 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.
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 (MALT-Type) 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.
Treatment and Management for Cutaneous B-Cell Lymphoma
Overtreatment in follicle center lymphoma (FCL) and marginal zone B-cell lymphoma (MZL) must be avoided because cutaneous B-cell lymphomas (CBCLs) have a much better prognosis than their nodal counterparts. Aggressive systemic treatment regimens are suitable only for diffuse large B-cell lymphoma (DLBCL) and for patients with extracutaneous spread. Treatment follows standard regimens for primary nodal lymphomas in these patients, as in B-cell lymphoma. The treatment of primary cutaneous lymphomas associated with an excellent prognosis depends on whether the patient has a solitary lesion or multiple lesions.
First-line treatments for solitary lesions include surgical excision, antibiotics, and radiotherapy. Antibiotics and radiotherapy, but not surgical excision, are also first-line treatments for multiple lesions. Antibiotic treatment consists of doxycycline at 100 mg twice daily for 3 weeks or pulse therapy with cefotaxime in Borrelia -associated cases. Radiotherapy may include the following:
- 20-100 kV Orthovolt therapy 1-4 times per week, to a total dose of up to 15-20 Gy
- Extended-field irradiation at 30 Gy (4 fractions of 2.5 Gy/wk)
- Localized-field irradiation as either a boost after extended-field irradiation or as definitive treatment
- Energies of 45 kV x-ray filtered by 0.55 aluminum for small-depth lesions and higher energies (≤100 kV) for deeper lesions; radiation dose varies from 30-40 Gy (fractions of 2 Gy/wk)
- Intralesional glucocorticosteroids
- Intralesional rituximab: Injections 2-3 times weekly; single doses of 1-3 mL of stock solution (10 mg mL-1) or 3 times a week with 3 mL of stem solution (at 10 mg/mL); therapy repeated 6 times every 26 days
- Intralesional interferon alfa: 3 and 9 million U subcutaneously (weekly basis) or 9 million U 3 times a week
- Intralesional interferon alfa: 3 and 9 million U subcutaneously (weekly basis) or 9 million U 3 times a week
- Intralesional rituximab: Injections 2-3 times weekly; single doses of 1-3 mL of stock solution (10 mg mL-1) or 3 times a week with 3 mL of stem solution (at 10 mg/mL); therapy repeated 6 times every 26 days
- Intravenous rituximab: 375 mg/m2 weekly for 4 weeks
- Radiotherapy: 20-100 kV Orthovolt therapy 1-4 times per week, to a total dose of up to 15-20 Gy ; extended-field irradiation at 30 Gy (4 fractions of 2.5 Gy/wk); localized-field irradiation as either a boost after extended-field irradiation or as definitive treatment; energies of 45 kV x-ray filtered by 0.55 aluminium for small depth lesions and higher energies (≤100 kV) for deeper lesions; radiation dose varies from 30-40 Gy (fractions of 2 Gy/wk)
- Surgical excisions
- Monochemotherapy: Liposomal doxorubicin or pegylated liposomal doxorubicin at 20-40 mg/m2 intravenously every 2-4 weeks
- Polychemotherapy: CHOP (cyclophosphamide, hydroxydaunorubicin, Oncovin [vincristine], and prednisone) regimen
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.