The treatment for cutaneous B-cell lymphoma (CBCL) is based on the histology (what the cells look like under the microscope), where the lesions are located on the body, and the size and number of lesions. Depending on these factors, the types of therapy chosen can vary from a “watch and wait” approach, skin directed therapy, or even in some cases systemic therapy. It’s important that the doctor treating your CBCL understands that CBCL is different than systemic (internal) B-cell lymphomas.
An important determinant of the best treatment for your CBCL is what type of CBCL you have. When a skin biopsy shows B-cell lymphoma it is very important to make sure that the lymphoma is truly coming from the skin, and not from a systemic lymphoma that has spread to the skin. Imaging tests such as CT (computerized axial tomography) and/or PET (positron emission tomography) scans are usually done to verify this. Bone marrow tests are sometime also done.
There are three main types of CBCL:
- Primary cutaneous follicle center lymphoma
- Primary cutaneous marginal zone lymphoma
- Primary cutaneous diffuse large B-cell lymphoma (including leg type)
Primary cutaneous follicle center lymphoma and primary cutaneous marginal zone lymphoma are together referred to as “indolent” (slow growing) types. In general, the indolent types are often treated locally, for example with radiation or surgery. When there are more widespread lesions or for primary cutaneous diffuse large B-cell lymphomas, systemic (internal) therapies such as rituximab or combination chemotherapy may be used. It’s common for the indolent CBCLs to recur over time, usually only on the skin.
For patients with primary cutaneous follicle center lymphoma or primary cutaneous marginal zone lymphoma who are not experiencing symptoms, treatment can be deferred. This approach is called “watchful waiting” or “watch and wait.” Laboratory and imaging tests are performed just as often as follow-up evaluations for active treatment would be. When and if the patient begins to develop symptoms or there are signs that the disease is progressing, active treatment is started.
The advantages of watch and wait are that patients do not experience the many possible side effects associated with anti-cancer treatments, which can negatively impact patients’ quality of life.
If only one or a few skin lesions are present, surgery can be performed to remove the lesions. Surgical removal of lesions is usually very effective. However, there is about a 50% likelihood that CBCL will reappear or a new spot will develop somewhere else.
Examples of topical therapies used to treat CBCL include topical corticosteroids, imiquimod, topical nitrogen mustard and bexarotene. There are limited studies done on the effectiveness of topical therapies for CBCL, but many lymphoma experts recommend them for the indolent CBCLs, and they are generally very safe and well-tolerated. Topical steroids are particularly helpful for managing symptoms, such as itching.
Radiation therapy is also highly effective, with most patients having a complete response (100% clearance of skin lesions) in the area treated. As with surgery, about one-half of patients will experience a recurrence of the tumor, usually in another location. Patients who have more than one lesion have a higher risk of recurrence than those with a single lesion. The dose of radiation used is often less than for other types of cancers, with a low impact on internal organs.
When indolent CBCL is present, a small amount of a drug — usually corticosteroid, interferon, or the antibody drug rituximab (Rituxan®) —can be injected directly into the lesion.
In the limited studies that have been done with intralesional therapies for indolent CBCL, patients did not experience adverse reactions except pain at the injection site. The rates of complete remission with this treatment are high and, in some cases, lesions other than the one injected showed regression. However, recurrences were also common.
Systemic (internal) therapies for CBCL may be used when there are widespread lesions, when there is spread outside the skin, when skin directed therapy isn’t feasible for cosmetic reasons, or in the case of primary cutaneous diffuse large-B cell lymphoma. The most common systemic therapies used for CBCLs are rituximab, combination chemotherapies such as CHOP, and combinations of rituximab and chemotherapy (“R-CHOP”).
Rituximab was the first monoclonal antibody cancer drug approved by the US Food and Drug Administration (FDA). Rituximab targets CD20, which is a molecule on the surface of both normal and abnormal B-cells, including most CBCLs.
Rituximab is the most common biologic drug used in lymphoma treatment regimens, either alone or in combination with standard chemotherapy regimens.
With systemic rituximab therapy, there is a drop in the number of normal B cells as well as a decrease in the malignant lymphoma cells. Common side effects include mild reactions during infusion, such as fevers or chills.
Multiagent chemotherapies used to treat systemic/internal B-cell lymphomas may be used for CBCL that is widespread or has spread outside the skin. The most common regimens used are CVP (also called COP) and CHOP.
CVP is a combination therapy consisting of cyclophosphamide (Cytoxan®), vincristine (Oncovin®), and prednisone.
One of the most common chemotherapy regimens for CBCL is called CHOP, which is a combination of Cytoxan® (cyclophosphamide), hydroxy doxorubicin (Adriamycin®), Oncovin® (vincristine), and prednisone.
Most patients with CBCL will respond to chemotherapy, but the rates of relapse are high.
Chlorambucil, CVP, and CHOP are often given in combination with rituximab (Rituxan®). The response rates of these treatments is slightly better than those of the chemotherapy regimens used without rituximab.
The possibility of treating PCMZL associated with B. burgdorferi has been considered. However, the efficacy of antibiotic treatment is poorly documented. Some patients reported achieving a response following an antibiotic regimen. There is some evidence to suggest that cephalosporins might be superior to high-dose tetracyclines; however, this is based on very limited experience from a few patients.