Siteman oncologists have long experience diagnosing and treating this disease. Cutaneous Lymphoma is a subset of non-Hodgkin lymphoma in which lymphocytes become malignant and affect the skin. Classification is based on lymphocyte type: B-lymphocytes (B-cell) or T-lymphocytes (T-cell).
Cutaneous T-cell lymphoma (CTCL) is the most common type of cutaneous lymphoma, representing about 80 percent of cases. It typically presents with red, scaly patches or thickened plaques of skin that often mimic eczema or chronic dermatitis. Progression from limited skin involvement is variable and may be accompanied by tumor formation, ulceration and exfoliation, complicated by itching and infections. Advanced stages are defined by involvement of lymph nodes, peripheral blood, and internal organs. Most CTCLs typically fall into the category of chronic lymphomas, which means they are treatable, but not curable. They are usually not life-threatening.
They are two main types of cutaneous T-cell lymphoma:
- Mycosis Fungoides goes through these stages: premycotic (scaly red patches on skin not exposed to sunlight), patch, plaque and tumor. Mycosis is the most common form of T-cell lymphoma.
- In the Sézary Syndrome, cancerous T-cells are also found in the blood. Skin all over the body may be red, itchy, peeling and painful. Researchers don’t know if this is a form of mycosis fungoides or a separate disease, but it is often referred to as the leukemia form of the disease.
Cutaneous B-cell lymphomas (CBCL) are a less common version of cutaneous lymphomas, making up about 20-25 percent of all cutaneous lymphomas. CBCLs are B-cell non-Hodgkin’s lymphomas which start in skin-based B-cells. Systemic or nodal B-cell lymphomas can secondarily involve the skin and when a skin biopsy shows B-cell lymphoma it is very important to make sure that the skin is the only organ involved and that it is not a systemic lymphoma presenting in the skin. The most common forms of CBCL are slow growing variations that respond well to mild treatments
Diagnosis of Cutaneous Lymphoma
To achieve a definitive diagnosis and differentiate it from other skin disorders, some of the tests that may be performed include:
- Skin biopsies with immunohistochemistry: Pathologists look for specific antigens like proteins in cancer cells to determine what antibody or immune therapy might be the best choice.
- TCR (T-cell receptor) gene rearrangement studies: This new tumor cell procedure improves the monitoring of diagnosis, staging, and follow-up in cutaneous T-cell lymphoma.
- Blood flow cytometry: By measuring the properties of cells in a sample of blood, this innovative test helps diagnose and classify certain cancers, such as lymphoma, and evaluate the risk of recurrence.
Skin-directed therapies include:
- Topical steroids to decrease inflammation of the skin
- Photodynamic UVA/UVB light therapy: This treatment uses special drugs, called photosensitizing agents, along with light to kill cancer cells. The drugs only work after they have been activated or “turned on” by certain kinds of light.
- Mechlorethamine: This topical is used to treat mycosis fungoides in patients who have received previous skin treatment. Part of a group of cancer medicines called alkylating agents, it interferes with the growth of cancer cells.
- Bexarotene gel: This retinoid cancer medication interferes with the growth and spread of cancer cells in the body to treat CTCL. It is usually given after other cancer medications have been tried without successful treatment.
- Localized radiation therapy: Cutaneous lymphomas can be highly radiosensitive, and radiation therapy is an important part of the treatment, either alone or as part of a combined approach.
- Extracorporeal photopheresis (ECP): This type of medical therapy used in patients with CTCL, involves drawing blood from the patient and separating out white-blood cells (WBCs) before the rest of the blood is returned to the patient. The WBCs are then mixed with a substance that makes the T-lymphocytes more sensitive to long-wavelength ultraviolet (UVA) light. The WBCs are exposed to the UVA which promotes death of the diseased cells before the treated WBCs are returned to the patient. The immune system recognizes the dying abnormal cells and begins to produce healthy lymphocytes to fight against those cells.
- Interferon alpha: Considered a targeted therapy, interferon stimulates the immune system to fight the cancer.
- Bexarotene capsules: The oral version may be given if the topical gel (above) doesn’t accomplish the intended effect.
- Vorinostat: This novel medication has been approved for the treatment of advanced CTCL. Its effect is to correct an imbalance in histones, the chief protein components in gene regulation. In studies, patients with CTCL treated with oral vorinostat had significant reductions in skin lesions and decreased disease progression.
- Romidepsin: This intravenous therapy is approved for patients with relapsed and/or refractory CTCL
- Methotrexate: This anti-metabolite agent is used in a variety of immune mediated diseases such as rheumatoid arthritis and psoriasis. It also interferes with folic acid metabolism in cancer cells. Low-dose methotrexate is a valuable first-line treatment for the majority of patients with early to intermediate-stage erythordermic CTCL, taken 1 day per week as single agent chemotherapy.
- Pralatrexate: This new anti-folate is FDA-approved for relapsed or refractory cutaneous T-cell lymphoma, given as a weekly cyclical intravenous infusion.
- Liposomal doxorubicin: This is a special formulation of doxorubicin, a drug that prevents cancer cells from growing by interfering with their DNA. A liposome is a microscopic sphere with layers of fat surrounding it. Giving doxorubicin in this form minimizes some of the side effects, and allows the drug to be more active. Doxorubicin is administered outpatient by intravenous infusion every 2-4 weeks.
- Gemcitabine: Mycosis fungoides (MF) patients may show resistance to conventional chemotherapy. After previous treatment, giving gemcitabine had a good response rate.
In treating cutaneous lymphomas, unlike most other cancers, physicians often use the same treatment repeatedly, such as light therapy and radiation. What worked once, often will work again. Patients with early stage disease can often achieve long-lasting remissions with only topical therapies. In CTCL, since malignant T-cells are thought to spend the majority of their time in the skin and are dependent upon the skin for survival, therapies aimed at the skin are likely to be effective for a long time. For more in-depth information on the disease, staging and treatment, check the National Cancer Institute pages.