Cutaneous lymphoma

Treatment

Seattle Cancer Care Alliance (SCCA) is one of few centers in the United States that has experience treating cutaneous lymphoma and specializes in providing novel therapies and clinical studies for this rare disease. Patients are referred to SCCA for our collaborative efforts between research and clinical studies for peripheral (systemic) and cutaneous T-cell lymphomas in general, as well as specific subtypes, bringing focus to this challenging disease.

SCCA's Cutaneous Lymphoma Specialty Clinic is a multidisciplinary clinic that brings together a team of specialists from dermatology, pathology, oncology, and radiation oncology to comprehensively evaluate and treat cutaneous lymphoma. Treatment options depend on the kind of lymphoma, including its location and stage, as well as other factors, such as overall health.

Clinical trial A type of research study that tests how well new medical approaches work in people. These studies test new methods of screening, prevention, diagnosis or treatment of a disease. Lymphoma Cancer that begins in the cells of the immune system. There are two basic categories of lymphomas: Hodgkin lymphoma and non-Hodgkin lymphomas. Cancer that begins in cells of the immune system. There are two basic categories of lymphomas. One is Hodgkin lymphoma, which is marked by the presence of a type of cell called the Reed-Sternberg cell. The other category is non-Hodgkin lymphomas, which includes a large, diverse group of cancers of immune system cells. Non-Hodgkin lymphomas can be further divided into cancers that have an indolent (slow-growing) course and those that have an aggressive (fast-growing) course. These subtypes behave and respond to treatment differently. Both Hodgkin and non-Hodgkin lymphomas can occur in children and adults, and prognosis and treatment depend on the stage and the type of cancer. Stage The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer and whether the cancer has spread from the original site to other parts of the body.
Dr. Ajay Gopal discusses treatment options for cutaneous lymphoma.

Treatment types

Several types of treatment can be used against skin lymphoma. These are generally divided into treatments directed at the skin and treatments that can affect the whole body (systemic treatments). Sometimes these two types of treatments are used together. In addition, because it is a chronic disease, many patients are treated with multiple therapies in their lifetime.

Many patients live normal lives while being treated for cutaneous lymphoma and some are able to remain in remission for long periods of time. It is important to discuss all of your treatment options as well as their possible side effects with your doctors to help make the decision that best fits your needs.

Lymphoma Cancer that begins in the cells of the immune system. There are two basic categories of lymphomas: Hodgkin lymphoma and non-Hodgkin lymphomas. Cancer that begins in cells of the immune system. There are two basic categories of lymphomas. One is Hodgkin lymphoma, which is marked by the presence of a type of cell called the Reed-Sternberg cell. The other category is non-Hodgkin lymphomas, which includes a large, diverse group of cancers of immune system cells. Non-Hodgkin lymphomas can be further divided into cancers that have an indolent (slow-growing) course and those that have an aggressive (fast-growing) course. These subtypes behave and respond to treatment differently. Both Hodgkin and non-Hodgkin lymphomas can occur in children and adults, and prognosis and treatment depend on the stage and the type of cancer. Remission A decrease in, or disappearance of, signs and symptoms of cancer. A decrease in, or disappearance of, signs and symptoms of cancer. In partial remission, some (but not all) signs and symptoms of cancer have disappeared. In complete remission, all signs and symptoms of cancer have disappeared, although cancer still may be in the body. Side effects A problem that occurs when treatment affects healthy tissues or organs. Some side effects of cancer treatment are nausea, vomiting, fatigue, pain, decreased blood cell counts, hair loss and mouth sores.
Recurrent (or relapsed) cutaneous lymphoma

Some lymphomas may not respond well to treatment. When a cancer returns after treatment it is called recurrent or relapsed. In general, cutaneous lymphomas come back in the skin. Additional treatments may be tried and may be effective for a time.

Treatments for mycosis fungoides and Sezary syndrome may help patients, sometimes for years, but they are rarely cured.

Patients with recurrent disease that is no longer responding to treatment may want to consider entering a clinical study.

Clinical trial A type of research study that tests how well new medical approaches work in people. These studies test new methods of screening, prevention, diagnosis or treatment of a disease. Relapse The recurrence (return) of disease after an apparent recovery.
Recurrent (or relapsed) cutaneous lymphoma

Some lymphomas may not respond well to treatment. When a cancer returns after treatment it is called recurrent or relapsed. In general, cutaneous lymphomas come back in the skin. Additional treatments may be tried and may be effective for a time.

Whole-body treatments

Systemic treatments, which enter the bloodstream and go throughout the body, are most useful for more advanced or aggressive disease. Systemic treatments include:

Photoimmune therapy (photopheresis)

Phtopheresis may be used for T cell lymphomas, especially Sezary syndrome. In this procedure your blood is pushed through a machine that separates and treats your lymphocytes with a drug and UV light. This method may kill some lymphoma cells and boost your immune system to kill other lymphoma cells. Photopheresis is typically well tolerated with relatively few side effects. The main side effect is sensitivity to sunlight for about one day after your treatment.

Inteferons

Interferons are substances that are produced by the immune system to help fight infections and a variety of cancers. They are administered by injection, typically into the abdomen or thigh, and can be combined with other therapies including photopheresis and retinoids. Potential side effects include flu-like symptoms, low blood counts, and auto-immune conditions, like thyroid problems.

Targeted therapies

These newer drugs that are designed to target specific parts of cancer cells or to boost your immune system to attack cancer cells. Targeted therapies used in treating cutaneous lymphomas include: vorinostat (Zolinza), romidepsin (Istodax), bortezomib (Velcade), denileukin diftitox (Ontak), rituximab (Rituxan), alemtuzumab (Campath). The side effects of these drugs are typically different, and often milder, than chemotherapy.

Retinoids

Used to treat a variety of skin diseases, retinoids may be given as a topical treatment or as a pill for more extensive lymphomas. Potential side effects depend on the type and dose of treatment and may include elevated blood sugar and/or fat levels, mood changes, thyroid problems, and eye problems. Retinoids may cause serious birth defects and should never be used by a woman who is pregnant or may become pregnant.

Chemotherapy

Not often used for cutaneous lymphoma, chemotherapy may be used when the disease is advanced or when other treatments are no longer working. It may also be useful when the disease has spread to the lymph nodes or other organs and tissues. It may be given alone or in combination with other chemotherapy or targeted drugs. Chemotherapies that may be used include: high dose methotrexate, pentostatin (Nipent), fludarabine (Fludara), liposomal doxorubicin (Doxil), gemcitabine (Gemzar), cyclophosphamide (Cytoxan), and a combination chemotherapy regimen known as CHOP (cyclophosphamide, adriamycin, vincristine, and prednisone).

There are a number of side effects associated with chemotherapy. The side effects vary greatly from person to person and depend on the type and dose of drug(s) given, how they are given, and the length of time they are taken. Some people experience few, if any, side effects. Your doctor may give you medicines to help protect your body’s normal cells and lessen the severity of side effects or prevent them before they happen. The time it takes to get over some side effects and regain energy depends on many factors, including your overall health and the drugs you were given. 

Chemotherapy Treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. It may be given alone or with other treatments. Treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. Chemotherapy may be given by mouth, injection, infusion or on the skin, depending on the type and stage of the cancer being treated. It may be given alone or with other treatments, such as surgery, radiation therapy or biologic therapy. Cyclophosphamide A drug used to treat many types of cancer. Cyclophosphamide damages the cell’s DNA and may kill cancer cells. It may also lower the body’s immune response. A drug used to treat many types of cancer and a certain type of kidney disease in children. Cyclophosphamide damages the cell’s DNA and may kill cancer cells. It may also lower the body’s immune response. Cyclophosphamide is a type of alkylating agent. Doxorubicin hydrochloride A drug that comes from the bacterium Streptomyces peucetius and is used to treat cancer. It damages the cell’s DNA, may kill cancer cells and blocks an enzyme needed for cell division and DNA repair. A drug that comes from the bacterium Streptomyces peucetius and is used alone or with other drugs to treat many types of cancer, including leukemia, lymphoma, neuroblastoma, sarcoma, Wilms tumor and certain cancers of the lung, breast, stomach, ovary, thyroid and bladder. It is also being studied in the treatment of other types of cancer. Doxorubicin hydrochloride damages the cell’s DNA and may kill cancer cells. It also blocks a certain enzyme needed for cell division and DNA repair. Doxorubicin hydrochloride is a type of anthracycline antibiotic and a type of topoisomerase inhibitor. Doxorubicin is the active ingredient of doxorubicin hydrochloride. Also called Adriamycin and hydroxydaunorubicin. Lymphocyte A type of immune cell that is made in the bone marrow and is found in the blood and in lymph tissue. The two main types of lymphocytes are B lymphocytes and T lymphocytes. A type of immune cell that is made in the bone marrow and is found in the blood and in lymph tissue. The two main types of lymphocytes (white blood cells) are B lymphocytes and T lymphocytes. B lymphocytes make antibodies, and T lymphocytes help kill tumor cells and help control immune responses. Lymphoma Cancer that begins in the cells of the immune system. There are two basic categories of lymphomas: Hodgkin lymphoma and non-Hodgkin lymphomas. Cancer that begins in cells of the immune system. There are two basic categories of lymphomas. One is Hodgkin lymphoma, which is marked by the presence of a type of cell called the Reed-Sternberg cell. The other category is non-Hodgkin lymphomas, which includes a large, diverse group of cancers of immune system cells. Non-Hodgkin lymphomas can be further divided into cancers that have an indolent (slow-growing) course and those that have an aggressive (fast-growing) course. These subtypes behave and respond to treatment differently. Both Hodgkin and non-Hodgkin lymphomas can occur in children and adults, and prognosis and treatment depend on the stage and the type of cancer. Photopheresis A procedure in which blood is removed from the body and treated with ultraviolet (UV) light and drugs that become active when exposed to light. The blood is then returned to the body. A procedure in which blood is removed from the body and treated with ultraviolet (UV) light and drugs that become active when exposed to light. The blood is then returned to the body. It is being studied in the treatment of some blood and bone marrow diseases and graft-vs-host disease (GVHD). Also called extracorporeal photopheresis. Prednisone A drug used to reduce inflammation and lower the body’s immune response. It is also used alone or with other drugs to prevent or treat many conditions. A drug used to reduce inflammation and lower the body’s immune response. It is used with other drugs to treat leukemia, lymphoma and mycosis fungoides (a type of skin lymphoma). It is also used alone or with other drugs to prevent or treat many other conditions. These include conditions related to cancer and its treatment, such as anemia (a low level of red blood cells) and allergic reactions. Prednisone is a type of therapeutic glucocorticoid. Side effects A problem that occurs when treatment affects healthy tissues or organs. Some side effects of cancer treatment are nausea, vomiting, fatigue, pain, decreased blood cell counts, hair loss and mouth sores. Side effects A problem that occurs when treatment affects healthy tissues or organs. Some side effects of cancer treatment are nausea, vomiting, fatigue, pain, decreased blood cell counts, hair loss and mouth sores. Symptom A physical or mental problem that a person experiences that may indicate a disease or condition. Symptoms cannot be seen and do not show up on medical tests. A physical or mental problem that a person experiences that may indicate a disease or condition. Symptoms cannot be seen and do not show up on medical tests. Some examples of symptoms are headache, fatigue, nausea and pain. T cell A type of white blood cell. T cells are part of the immune system and develop from stem cells in the bone marrow. They help protect the body from infection and may help fight cancer. A type of white blood cell. T cells are part of the immune system and develop from stem cells in the bone marrow. They help protect the body from infection and may help fight cancer. Also called T lymphocyte and thymocyte. Targeted therapy A type of treatment that uses drugs or other substances to identify and attack specific types of cancer cells while causing less harm to normal cells. A type of treatment that uses drugs or other substances to identify and attack specific types of cancer cells while causing less harm to normal cells. Some targeted therapies block the action of certain enzymes, proteins or other molecules involved in the growth and spread of cancer cells. Other types of targeted therapies help the immune system kill cancer cells, or they deliver toxic substances directly to cancer cells and kill them. Targeted therapy may have fewer side effects than other types of cancer treatment. Most targeted therapies are either small molecule drugs or monoclonal antibodies. Vincristine Vincristine is the active ingredient in vincristine sulfate, a drug used to treat acute leukemia and sometimes used with other drugs to treat lymphoma, rhabdomyosarcoma, neuroblastoma and Wilms tumor. Vincristine is the active ingredient in vincristine sulfate, a drug used to treat acute leukemia and sometimes used with other drugs to treat Hodgkin lymphoma, non-Hodgkin lymphoma, rhabdomyosarcoma, neuroblastoma and Wilms tumor. It is also being studied in the treatment of other types of cancer. Vincristine sulfate stops cancer cells from growing and dividing and may kill them. It is a type of vinca alkaloid. The brand name Oncovin has been taken off the market and is no longer available.
Whole-body treatments

Systemic treatments, which enter the bloodstream and go throughout the body, are most useful for more advanced or aggressive disease. 

High-dose chemotherapy with bone marrow transplant (BMT)

BMT may be used to treat cutaneous lymphoma on rare occasions. It is typically used only when other treatments are no longer working. This therapy uses high doses of chemotherapy and/or radiation to destroy both the healthy and cancerous blood cells in the bone marrow. Stem cells are then transplanted to travel to the marrow and begin making new blood cells. A bone marrow transplant is a complex treatment, with a number of potential complications and side effects.

Bone marrow The soft, spongy material in the center of your bones that produces all your blood cells, such as white blood cells, red blood cells and platelets. Bone marrow transplant The process of treating disease with high doses of chemotherapy, radiation therapy or both. Bone marrow or peripheral blood stem cells are given after treatment to help the body make more blood cells. The process of treating disease with high doses of chemotherapy, radiation therapy or both. Because this treatment destroys the bone marrow’s ability to produce blood cells, bone marrow or peripheral blood stem cells are given after treatment to help the body make more blood cells. Bone marrow transplant The process of treating disease with high doses of chemotherapy, radiation therapy or both. Bone marrow or peripheral blood stem cells are given after treatment to help the body make more blood cells. The process of treating disease with high doses of chemotherapy, radiation therapy or both. Because this treatment destroys the bone marrow’s ability to produce blood cells, bone marrow or peripheral blood stem cells are given after treatment to help the body make more blood cells. Chemotherapy Treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. It may be given alone or with other treatments. Treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. Chemotherapy may be given by mouth, injection, infusion or on the skin, depending on the type and stage of the cancer being treated. It may be given alone or with other treatments, such as surgery, radiation therapy or biologic therapy. Lymphoma Cancer that begins in the cells of the immune system. There are two basic categories of lymphomas: Hodgkin lymphoma and non-Hodgkin lymphomas. Cancer that begins in cells of the immune system. There are two basic categories of lymphomas. One is Hodgkin lymphoma, which is marked by the presence of a type of cell called the Reed-Sternberg cell. The other category is non-Hodgkin lymphomas, which includes a large, diverse group of cancers of immune system cells. Non-Hodgkin lymphomas can be further divided into cancers that have an indolent (slow-growing) course and those that have an aggressive (fast-growing) course. These subtypes behave and respond to treatment differently. Both Hodgkin and non-Hodgkin lymphomas can occur in children and adults, and prognosis and treatment depend on the stage and the type of cancer. Side effects A problem that occurs when treatment affects healthy tissues or organs. Some side effects of cancer treatment are nausea, vomiting, fatigue, pain, decreased blood cell counts, hair loss and mouth sores. Stem cell A cell from which other types of cells develop. For example, blood cells develop from blood-forming stem cells.
High-dose chemotherapy with bone marrow transplant (BMT)

BMT may be used to treat cutaneous lymphoma on rare occasions. It is typically used only when other treatments are no longer working. 

Skin directed treatments

In many cases, initial treatment of skin lymphoma involves treating the skin tumors directly, while trying to avoid harmful side effects on the rest of the body. Skin-directed treatments include:

Surgery

Surgery may be used to obtain a biopsy sample or, when there are few lesions, to completely remove the lesion(s). It is usually combined with other therapies.

Radiation therapy

Radiation therapy uses high-energy rays to kill cancer cells. Electron beam radiation is used most often for cutaneous lymphomas. The electrons only penetrate the skin, minimizing side effects to other organs and tissues. The possible side effects include skin irritation (sunburn-like symptoms, itching, dry skin) and fatigue.

Phototherapy

Phototherapy uses ultraviolet (UV) light to kill cancer cells in the skin. It may be combined with a drug, psoralen, that increases the activity of the UV light. This treatment, called PUVA, is given about three times per week. Potential side effects include nausea (from psoralen), sensitivity to sunlight, and sunburn-like symptoms.

Topical Medicines

Topical medicines, including steroids, chemotherapies, retinoids, and immune therapies, are applied directly to the skin. When applied topically to the skin, the effects are concentrated on the spot where applied minimizing side effects to other areas of the body. Possible side effects may occur in the area where the medicine is applied. The side effects depend on the type of topical therapy and may include thinning of the skin, skin irritations (rashes, itching), bruising easily, and dilated blood vessels.

Biopsy The removal of a sample of tissue or fluid that is examined to see whether cancer is present. This may be done with a large needle or through surgical removal of tissue or fluids. Lesion An area of abnormal tissue. A lesion may be benign (not cancer) or malignant (cancer). Lymphoma Cancer that begins in the cells of the immune system. There are two basic categories of lymphomas: Hodgkin lymphoma and non-Hodgkin lymphomas. Cancer that begins in cells of the immune system. There are two basic categories of lymphomas. One is Hodgkin lymphoma, which is marked by the presence of a type of cell called the Reed-Sternberg cell. The other category is non-Hodgkin lymphomas, which includes a large, diverse group of cancers of immune system cells. Non-Hodgkin lymphomas can be further divided into cancers that have an indolent (slow-growing) course and those that have an aggressive (fast-growing) course. These subtypes behave and respond to treatment differently. Both Hodgkin and non-Hodgkin lymphomas can occur in children and adults, and prognosis and treatment depend on the stage and the type of cancer. Radiation therapy The use of high-energy radiation from X-rays, gamma rays, neutrons, protons and other sources to kill cancer cells and shrink tumors. The use of high-energy radiation from x-rays, gamma rays, neutrons, protons and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body near cancer cells (internal radiation therapy or brachytherapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that travels in the blood to tissues throughout the body. Side effects A problem that occurs when treatment affects healthy tissues or organs. Some side effects of cancer treatment are nausea, vomiting, fatigue, pain, decreased blood cell counts, hair loss and mouth sores. Steroid A type of drug used to relieve swelling and inflammation. Some steroid drugs may also have antitumor effects. Symptom A physical or mental problem that a person experiences that may indicate a disease or condition. Symptoms cannot be seen and do not show up on medical tests. A physical or mental problem that a person experiences that may indicate a disease or condition. Symptoms cannot be seen and do not show up on medical tests. Some examples of symptoms are headache, fatigue, nausea and pain.
Skin directed treatments

In many cases, initial treatment of skin lymphoma involves treating the skin tumors directly, while trying to avoid harmful side effects on the rest of the body.