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Treatment

How Is Cutaneous T-Cell Lymphoma Treated?

Several options are available for treating the different types of cutaneous T-cell lymphoma. These options include both skin-directed treatments and systemic treatments. These treatments are sometimes used in combination with each other.

 

Treatments for Mycosis Fungoides

Treatments for mycosis fungoides include both skin-directed and systemic treatments.

 

Skin-Directed Treatments for Mycosis Fungoides

For patients with early-stage mycosis fungoides, treatments are aimed directly at the skin rather than the entire body. Skin-directed treatments for mycosis fungoides include:

Phototherapy with Ultraviolet (UV) Light

Phototherapy uses two types of ultraviolet (UV) light to destroy cancer cells: ultraviolet A (UVA) or ultraviolet B (UVB). Both types of UV light are administered using fluorescent lamps specially calibrated to maximize the effect of treatment while minimizing the risk of burns. Phototherapy treatments are usually given twice a week for UVA and three times a week for UVB. If UVA light is used, the patient is first given drugs called psoralens. The combination of UVA light and psoralens is called PUVA. Approximately two hours before treatment, the patient takes psoralens orally as a pill. This allows the drugs time to circulate throughout the body. The UVA light activates the drugs, destroying the cells the drugs came into contact with. Psoralens can cause nausea in some patients. Treatment with PUVA can cause increased sensitivity to sunlight. This can increase a person’s risk of severe skin burns and cataracts. For this reason, it is very important for patients treated with PUVA to take measures to protect themselves from the sun in the two days immediately following treatment. This type of treatment can also increase a person’s risk of developing skin cancer in the future, so a patient’s total number of light treatments is limited to a predetermined maximum number of sessions. UVB light is typically used to treat thinner skin lesions. Treatment with UVB light does not require additional drugs.

 

Topical Medicines

Topical medicines are applied directly to the skin. The effects of these medicines are strongest in the area where they are applied. Types of topical medicines include:

Topical Chemotherapy

Topical chemotherapy are medications to kill cancer cells given as a cream or an ointment applied to the skin. Treatment with these drugs can result in irritation, redness, or swelling in the treated area. The long-term risk for developing other types of skin cancer is higher for patients being treated with these drugs in the area where the drugs are applied.

Topical Corticosteroids

Cortisol is a naturally-occurring hormone in the body that can affect immune cells. Corticosteroids can be given orally (as a pill) or topically (as a cream, gel, or ointment). Because topical corticosteroids are applied directly to the skin and not circulated throughout the body, patients being treated with them may experience fewer side effects.

Topical Retinoids

Retinoids are drugs connected to vitamin A and affect certain genes involved in the growth and maturation of cancer cells. Some retinoids can be applied directly to skin lesions as a gel. Treatment with topical retinoids can result in irritation, itching, and redness in the area where the drugs are applied. They can also cause increased sensitivity to sunlight in the treated area. Women who are pregnant or could become pregnant should not be given topical retinoids, as the drugs can cause serious birth defects.

Topical Immune Therapy

Imiquimod is a type of topical immune therapy that comes in the form of a cream. When imiquimod is applied to skin lesions, it causes the immune system to react in such a way that may destroy cancer cells. Though most often used to treat other types of skin cancer, imiquimod can also be used to treat early-stage cutaneous T-cell lymphoma. Possible side effects for imiquimod include irritation, itching, and redness in the treated area.

Radiation Therapy

Radiation uses special equipment to deliver high-energy particles, such as x-rays, gamma rays, electron beams or protons, to kill or damage cancer cells. Radiation (also called radiotherapy, irradiation, or x-ray therapy) can be delivered internally through seed implantation or externally using linear accelerators (called external beam radiotherapy, or EBRT). Radiation may be used as a solitary treatment or with surgery and/or chemotherapy. The equipment used to deliver the radiation therapy is called a linear accelerator. The linear accelerator has a moveable arm, which enables the radiation to be focused on the part of your body where the cancer is located. Developments in EBRT equipment have enabled physicians to offer conformal radiation. With conformal radiation, computer software uses imaging scans to map the cancer three-dimensionally. The radiation beams are then shaped to conform, or match, the shape of the tumor.

Radiation works by breaking a portion of the DNA of a cancer cell, which prevents it from dividing and growing. Radiation therapy can be systemic, meaning it moves throughout your bloodstream. Systemic therapies are usually given as an injection into a blood vessel or are taken as a pill. Systemic treatments expose your entire body to cancer-fighting medication. Radiation therapy is typically given as a "local" treatment however, meaning it affects only the part of the body that needs therapy.

 

Systemic Treatments for Mycosis Fungoides

Although mycosis fungoides may be confined to the skin for many years, it can sometimes cause widespread involvement with significant symptoms that may be resistant to skin-directed therapies. In some cases, the disease can spread internally to the lymph nodes or other organs.

Thus, systemic treatments, which affect the entire body, may be necessary. Systemic treatments for mycosis fungoides include:

Oral Retinoids

Taken by mouth as a pill, oral retinoids are drugs connected to vitamin A that affect certain genes in cancer cells that cause them to grow and mature. The drugs are typically well tolerated but can cause increased blood levels of triglycerides (fats) and problems with the thyroid and eyes. Women who are pregnant or could become pregnant should not take oral retinoids, as the drugs can cause serious birth defects.

Photopheresis

Photopheresis (also called photoimmune therapy or extracorporeal photopheresis) treats cutaneous cancer in two ways. It directly attacks cells and increases the body’s immune system response against them. Blood is removed from the body and put into a special machine that separates the lymphocytes from the rest of the blood. After being treated with PUVA (see above), the lymphocytes are mixed back in with the rest of the blood, which is infused back into the patient. Treatments are usually given two days in a row, with each procedure generally lasting a few hours. Treatments are typically repeated every four weeks.

Chemotherapy

Chemotherapy is a medication or combination of medications used to treat cancer. Chemotherapy can be given orally (as a pill) or injected intravenously (IV). When chemotherapy drugs enter the bloodstream, they destroy cancer cells. Chemotherapy is particularly useful for cancers that have metastasized, or spread. Chemotherapy attacks all quickly-dividing cells, regardless of whether they are cancerous which can cause a number of side effects, including hair loss, mouth sores, loss of appetite, nausea and vomiting, diarrhea, and low blood counts. Low blood counts can increase a patient’s risk of infection, bruising or bleeding, fatigue, and shortness of breath. The side effects of chemotherapy are generally temporary and often go away once treatment is completed. Chemotherapy regimens vary from patient to patient. They are generally repeated several times in cycles, with three to four weeks separating each cycle to allow damaged normal cells time to recover. After the first two or three sessions of chemotherapy, patients may have a CT or PET scan to see if the drug(s) is effective. If the drug(s) is not working, it may be switched out for a new drug(s).

 

Although these treatments can help patients control cutaneous lymphoma for many years, they rarely cure the disease. Stem cell transplantation (see below) may be an option for those patients no longer responding to other types of treatment and may be the only chance for a possible cure.

Targeted Therapy

Targeted therapy is a type of cancer treatment that uses drugs or other substances to precisely identify and attack cancer cells. Usually, targeted therapy does less damage to normal cells than other cancer treatments.  

Stem Cell Transplant

A stem cell transplant is a procedure that uses stem cells to replace blood cells in the bone marrow that have been damaged or destroyed by chemotherapy, radiation or disease. For patients receiving a stem cell transplant, the first step is to lower the amount of cancer present in the patient’s body by induction treatment. After induction treatment, stem cells are harvested from the patient’s blood or bone marrow. Once harvested, the cells are frozen. After the stem cells have been removed, the patient receives high-dose chemotherapy to kill any leftover myeloma cells. The treatment also kills all of the remaining normal bone marrow cells. The frozen stem cells are then thawed and given back to the patient through an intravenous line in a process similar to a blood transfusion. The stem cells travel to the bone marrow where they begin to make new blood cells.

BMC specializes in autologous stem cell transplants, in which a patient’s own stem cells are used as opposed to a donor’s (this kind of transplant is called an allogeneic transplant). Autologous stem cell transplants are relatively safe and have a low risk of serious complications. For patients who have an autologous stem cell transplant, recurrence (the cancer returning) is common.

Autologous transplants do not cure cutaneous lymphoma, and certain problems, such as high fevers from infections, mouth sores, cramps, diarrhea, and low blood and platelet counts, may occur. These side effects can be treated with IV antibiotics, red blood cell and platelet transfusions, and other medications to relieve symptoms.

The Autologous Stem Cell Transplant Program at BMC provides a multidisciplinary team approach for patients undergoing high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation. The program maintains accreditation through the Foundation for the Accreditation of Cellular Therapy (FACT).

 

Treatments for Sezary Syndrome

Skin-directed treatments are not typically used to treat Sezary syndrome because the disease involves both the skin and the blood at the time of diagnosis, making this particular type of treatment less effective. Many of the systemic treatments used to treat advanced mycosis fungoides, such as photopheresis, oral retinoids, chemotherapy, and targeted therapy, are also used to treat Sezary syndrome. Chemotherapy or targeted therapy is typically used to treat patients who are no longer responding to other forms of treatment. A stem cell transplant might also be a possibility for patients for whom other forms of treatment are no longer effective.

 

Treatments for Lymphomatoid Papulosis

Immediate treatment for lymphomatoid papulosis is unnecessary if the disease is at a low-level involvement and isn’t causing the patient any symptoms. If treatment is necessary, the most common options for patients with only a few skin lesions are phototherapy and topical corticosteroids. Systemic treatments, such as oral retinoids or low-dose methotrexate (a chemotherapy drug), can be used to treat patients with more extensive skin involvement.

 

Treatments for Primary Cutaneous Anaplastic Large Cell Lymphoma

Physicians commonly use surgery or radiation therapy to treat single or small groups of lesions. Systemic medicines, such as methotrexate, retinoids, or targeted therapy, can be used to treat cases of C-ALCL that involve more skin. Systemic chemotherapy is often used to treat C-ALCL that has spread internally.

 

Treatments for Subcutaneous Panniculitis-Like T-Cell Lymphoma

The prognosis (outlook) for subcutaneous panniculitis-like T-cell lymphoma is generally excellent. Patients with the disease are able to live a long time. The disease can often be controlled for an extended period of time using just corticosteroids. Chemotherapy and radiation therapy have also been used to successfully treat the disease.

 

Treatments for Primary Cutaneous Peripheral T-Cell Lymphoma, Unspecified

Primary Cutaneous Aggressive Epidermotropic CD8+ Cytotoxic T-Cell Lymphoma

Physicians use systemic therapies to treat this fast-growing type of lymphoma.

 

Primary Cutaneous Gamma/Delta T-Cell Lymphoma

Physicians treat primary cutaneous gamma/delta T-cell lymphoma using systemic chemotherapy or radiation therapy. This fast-growing type of lymphoma tends to behave aggressively.

 

Primary Cutaneous CD4+ Small/Medium Pleomorphic T-Cell Lymphoma

This type of lymphoma can be treated in several ways. If only a single tumor or a few lesions are present, as is often the case, surgery can be used to remove the tumor, or it can be treated with radiation therapy. The prognosis for this particular type of lymphoma is typically good, especially if only a single tumor is present.

 

Primary Cutaneous Peripheral T-Cell Lymphoma, Unspecified

This type of lymphoma can be treated using systemic chemotherapy with some success. However, the disease can be very difficult to treat if it recurs.

 

How Is Cutaneous B-Cell Lymphoma Treated?

Most cases of primary cutaneous B-cell lymphoma are slowly progressive conditions that can be managed by local injection of steroids into the lesions or localized radiation therapy. When the lesions are widespread, targeted therapy that is given as an IV infusion is often required.

Primary cutaneous diffuse large B-cell lymphoma of the leg is typically an aggressive condition and is usually managed with a combination of chemotherapy with or without targeted therapy.

 

Our Team

BMC’s comprehensive cutaneous lymphoma team includes physicians who work in dermatology, dermatopathology, hematology, medical oncology, radiation oncology, and surgical oncology. The team’s patient-centered, multidisciplinary approach assures each patient benefits from the collaborative expertise of physicians uniquely focused on their individual needs.

 

Cancer Clinical Trials

BMC offers a number of clinical trials specifically for patients with cutaneous lymphoma. Promising new techniques in the diagnosis, treatment, and care of patients with cancer are tested in these studies. The number and types of clinical trials available are constantly changing. View an up-to-date list of ongoing trials here. Those interested in participating in any clinical trials at BMC should talk with their physician.

 

Cancer Support Services

A diagnosis of cancer can be a life-altering experience. The team at Boston Medical Center understands that cancer affects patients and their loved ones in many ways. The team is here to support patients during all aspects of care—from diagnosis through treatment and recovery. The Cancer Support Services Program is dedicated to providing patients and their loved ones with a comprehensive set of services that address a wide range of needs. Services include:

  • Cancer support/discussion groups
  • Support activities
  • Special programs and events
  • Complementary services

To learn more, see the most recent quarterly newsletter and event calendar or visit Cancer Support Services.

 

Additional Support

Patient Navigators

Patients have the option of working with a patient navigator who may be able to provide support services related to their care. Patient navigators frequently assist with:

  • Appointment reminders
  • Helping patients understand complex treatment schedules
  • Transportation
  • Insurance questions
  • Interpreter services
  • Connecting patients to Cancer Support Services programs and events
  • Referrals to community resources

 

Social Workers

Social workers are available to provide additional support services to patients and families and to help with emotional, psychological, and social service needs. They are available on inpatient and outpatient units. Social workers can meet with patients before or after their other appointments. Patients can discuss these options with their physician or ask for a referral.

 

Nutritionists

Patients who have concerns about their diet and nutrition during the course of their cancer treatment can meet with one of BMC’s nutritionists to discuss their dietary concerns. Nutritionists are also available to meet with patients before or after their other appointments. Patients can talk with their physician or social worker if they are interested in seeing a nutritionist.

References

American Cancer Society. 2014. Lymphoma of the Skin. PDF.
Cutaneous Lymphoma Foundation. 2012. A Patient’s Guide to Understanding Cutaneous Lymphoma. PDF.
Lymphoma Association. 2013. Cutaneous B-cell lymphoma
Lymphoma Research Foundation. 2013. Getting the Facts: Cutaneous T-Cell Lymphoma (CTCL). PDF.

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