WHAT IS LYMPHOMA?
Circulating through the lymphatic system is a colourless fluid called lymph. This contains cells known as lymphocytes. These are a type of white blood cell and are an essential part of the body's defence against infection and disease. The lymphatic system is a complex system made up of organs such as the bone marrow, the thymus, the spleen, and the lymph nodes (or lymph glands), which are connected by a network of tiny lymphatic vessels. Lymph nodes are found all over the body.
There are two main types of lymphocyte: B cells and T cells. Most lymphocytes start growing in the bone marrow. The B cells continue to develop in the bone marrow, whereas the T cells go from the bone marrow to the thymus gland (behind the breast bone) and mature there. Once they are mature, both B cells and T cells help us fight infections. Lymphoma is a growth of one cell that becomes a huge population identical to itself and it’s a cancer of these lymphocytes which are part of our immune system and of our white blood cells.
There are more than 20 different types of Non-Hodgkin's lymphoma which is a cancer of the lymphatic system and Cutaneous T cell lymphoma (CTCL) is a type of this. CTCL is a rare type, making up about 5% of all cases. It is a cancer of the T-lymphocytes and most often occurs in people aged between 40 and 60. It is not infectious.
Unlike other forms of NHL, CTCL mainly affects the skin. It is caused by the uncontrolled growth of a type of white blood cell within the skin, called a T-cell which become malignant. The causes are not known.
Mycosis Fungoides is a type of CTCL when the blood is not affected. Sezary syndrome is a specific type of CTCL in which large areas of skin or lymph glands are affected and abnormal T lymphocytes are also found in the blood.
WHAT IS CTCL?
The earliest name for skin lymphoma was mycosis fungoides, first described early in the 19th century because the first patient diagnosed had skin tumors that looked like mushrooms. They did not know about lymphocytes until the middle of the 20th century, so the whether the name is suitable or not is still causing some debate. Cutaneous T-cell lymphoma (CTCL) is the name used to describe a number of diseases in which some of the T-lymphocytes in the skin become malignant and thus affect the skin. Mycosis Fungoides is one form of this.
HOW COMMON IS CTCL?
It is an extremely rare disease, with an incidence of about 2 per million people. This represents about 1000 new cases in the USA per year. Although figures published show that men are twice as likely as women to have the disease, that it is several times more common in those of African as compared to European or Asian descent and that the average age at diagnosis is 50 years with children are rarely affected, researchers are questioning these statistics. However, as medical technology and communications such as the internet continue to improve, more cases are probably being diagnosed than say, ten years ago, so it could conceivably be more common than is known at this time.
WHAT DOES IT LOOK LIKE?
Cutaneous T-cell lymphoma usually develops slowly over many years and is not usually immediately diagnosed. This is because it doesn't look the same for all patients. It can begin as a little pink area on your skin or a little dry area on your skin, or a scaly spot. It can begin with tumors or big nodules that come up. It can begin as redness. It can begin as white patches. There are many different ways it can begin. Patches, plaques and tumours are the clinical names of the different presentations. Patches are usually flat, possibly scaly and look like a "rash." CTCL patches are often mistaken for eczema, psoriasis or non-specific dermatitis. Plaques are thicker, raised lesions. Tumors are raised "bumps" which may or may not ulcerate. A common characteristic is itching, although many patients do not experience itching. It is possible to have one or all three of these phases. Dark skinned people with CTCL often have patches or plaques which are paler than the surrounding skin, which is called hypopigmentation.
IS IT EASY TO DIAGNOSE?
No. One study has indicated that the average time before diagnosis is made is six years. Some people have had the disease for many years (far longer than six) and have only shown one presentation, such as a spot resembling a pimple, or a rash, or an area of dry skin. Cutaneous T-cell lymphoma may mimic common skin disorders such as eczema, psoriasis or contact dermatitis, so confusion with benign conditions is common. Patients are then treated for these disorders and only when the condition worsens or does not respond to treatment do doctors then look further. Biopsies taken from the skin are the only accepted method of diagnosing MF. A person may have a few patches and biopsies may not show anything, but the advice now given is to obtain more when the skin condition does not improve, taking from different areas. The chance of recovery (prognosis) and choice of treatment depend on the stage of the cancer (whether it is just in the skin or has spread to other places in the body) and the patient's general state of health. There are several other types of cancer that start in the skin. The most common are basal cell cancer and squamous cell cancer, while another type of skin cancer called melanoma is becoming more prevalent, particularly in hot, sunny countries where people frequently expose their bodies to the sun. However, CTCL is NOT a cancer of the skin - it is a lymphoma (cancer of the blood).
STAGES OF CTCL and ACCEPTED TREATMENTS FOR EACH STAGE:
Note - each medical practitioner may opt for his/her preferred treatment. This is not to say that any are incorrect. Therefore, the term "Usual treatments" is used, but there are obviously variations. Stage IA - Patches: The skin has dry, red, scaly patches, but no tumours. Less than 10% of the skin surface area (T1 skin disease) is involved. The lymph nodes are not larger than normal. Low Risk And/or Plaques which are raised and are well-demarcated, which may have a fine scale, may be multiple or single. In dark-skinned individuals, the patches may appear as hypopigmented or hyperpigmented areas. Patches and plaques may affect any area of the skin, but they often are distributed asymmetrically in the areas that a bathing suit would cover (ie, hips, buttocks, groin, lower trunk, axillae, breasts). When the disease affects the scalp, it often is accompanied by alopecia.
USUAL TREATMENTS
Treatment may be one of the following:
1. Phototherapy (PUVA therapy) with or without biological therapy.
2. Total skin electron beam radiation therapy (TSEB radiation therapy).
3. Topical chemotherapy.
4. Local electron beam or x-ray therapy to reduce the size of the tumor or to relieve symptoms.
5. Clinical trials of phototherapy.
6. Interferon alpha (biological therapy) alone or in combination with topical therapy.
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Stage IB disease is defined as patchy or plaquelike skin disease involving greater than or equal to 10% of the skin surface area (T2 skin disease). Low Risk
USUAL TREATMENTS
Treatment may be one of the following:
1. Phototherapy (PUVA therapy) with or without biological therapy.
2. Total skin electron beam radiation therapy (TSEB radiation therapy).
3. Topical chemotherapy.
4. Local electron beam or x-ray therapy to reduce the size of the tumor or to relieve symptoms.
5. Clinical trials of phototherapy.
6. Interferon alpha (biological therapy) alone or in combination with topical therapy.
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Stage 11A
Either of the following may be true: The skin has red, dry, scaly patches, but no tumours. Lymph nodes are larger than normal, but do not contain cancer cells. There are tumours on the skin. The lymph nodes are either normal or are larger than normal, but do not contain cancer cells. (T1-2 skin disease) Low Risk
USUAL TREATMENTS
Treatment may be one of the following:
1. Phototherapy (PUVA therapy) with or without biological therapy.
2. Total skin electron beam radiation therapy (TSEB radiation therapy).
3. Topical chemotherapy.
4. Local electron beam or x-ray therapy. 5. Interferon alpha (biological therapy) alone or in combination with topical therapy.
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Stage IIB disease is defined by the presence of tumors (T3 skin lesions). Skin tumors Patients with evidence or a history of patchy or plaquelike skin lesions also can have tumors. Tumors are red-violet nodules that may be dome-shaped, exophytic, or ulcerated. Intermediate Risk
USUAL TREATMENTS
Treatment may be one of the following:
1. Phototherapy (PUVA therapy) with or without biological therapy.
2. Total skin electron beam radiation therapy (TSEB radiation therapy).
3. Topical chemotherapy.
4. Local electron beam or x-ray therapy.
5. Interferon alpha (biological therapy) alone or in combination with topical therapy Stage III disease is defined by the presence of generalized erythroderma which means that nearly all of the skin is red, dry, and scaly. The lymph nodes are either normal or are larger than normal, but do not contain cancer cells. The skin can thicken in the folds of the face and palms and soles can be cracked. Intermediate Risk
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USUAL TREATMENTS
Treatment may be one of the following:
1. Phototherapy (PUVA therapy) with or without biological therapy.
2. Total skin electron beam radiation therapy (TSEB radiation therapy).
3. Topical chemotherapy.
4. Local electron beam or x-ray therapy.
5. Systemic chemotherapy with or without therapy to the skin.
6. Chemotherapy for mycosis fungoides and Sezary syndrome.
7. Extracorporeal photochemotherapy.
8. Interferon alpha (biological therapy) alone or in combination with topical therapy.
9. Retinoids.
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Stage IVA
The skin is involved, in addition to either of the following: Cancer cells are found in the lymph nodes. Cancer has spread to other organs, such as the liver or lung. High Risk Stage IVB disease is defined by the presence of visceral involvement (eg, liver, lung, bone marrow). High Risk
USUAL TREATMENTS
Treatment may be one of the following:
1. Systemic chemotherapy.
2. Topical chemotherapy.
3. Total skin electron beam radiation therapy (TSEB radiation therapy).
4. Phototherapy (PUVA therapy) with or without biological therapy.
5. Local electron beam or x-ray therapy.
6. Chemotherapy for mycosis fungoides and Sezary syndrome.
7. Extracorporeal photochemotherapy 8. Interferon alpha (biological therapy) alone or in combination with topical therapy.
9. Monoclonal antibody therapy.
10. Retinoids.
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Recurrent:
Recurrent disease means that the cancer has come back after it has been treated. It may come back where it started or in another part of the body. Treatment depends on many factors, including the type of treatment the patient received before. Depending on the patient's condition, treatment may be one of the following:
1. Local electron beam or x-ray therapy.
2. Total skin electron beam radiation therapy (TSEB radiation therapy).
3. Phototherapy (PUVA therapy).
4. Topical chemotherapy.
5. Systemic chemotherapy.
6. Extracorporeal photochemotherapy.
7. Clinical trials of biological therapy.
8. Clinical trials of bone marrow transplantation.
TREATMENT OPTIONS
The choice of treatments differ from country to country and from doctor to doctor. Not all the treatments mentioned below may be available to those who do not live in the USA. For a full listing, this is available on the MF Foundation site: http://www.mffoundation.org/CTCL_treatments.html From their preface and a listing of available treatments:
CTCL Treatment Options
The following information attempts to provide patients and their family members with an explanation of the available treatments for CTCL. It is not a comprehensive listing, but rather lists treatments that are often considered and used for CTCL. The information provides summaries of treatments and should not substitute for a thorough discussion with an experienced physician about which treatment is best for an individual. The treatments are organized in the following manner:
Skin-directed therapies:
Topical therapies:
Bexarotene
Topical carmustine
Topical mechlorethamine (nitrogen Mustard
Topical steroids
Phototherapy:
PUVA phototherapy
Narrow band UVB phototherapy
UVB phototherapy Radiation therapy:
Local X-ray therapy
Local and total skin electron beam radiation therapy
Systemic therapies:
Biological / Immuno- therapies:
Bexarotene (Targretin®) Capsules.
Denileukin Diftitox (Ontak®)
Interferon Photopheresis Alemtuzumab (Campath-1H®)
Chemotherapies:
Methotrexate Pentostatin & other purine analogues (Fludarabine, 2- deoxychloroadenosine
Liposomal doxorubicin (Doxil®)
Gemcitabine (Gemzar®)
Cyclophosphamide, oral
Combination chemotherapy
Bone marrow / Stem cell transplantation:
Bone marrow/Stem cell Transplantation
CLINICAL TRIALS
There are many of these which are ongoing, including a variety of drugs, procedures and vaccines.
WHAT DOES THE FUTURE HOLD FOR ME?
If the cancer remains in the skin, the chances of having a normal lifespan are excellent. Mycosis Fungoides cannot be cured, but the disease can be controlled via the treatments mentioned above. The cancer can go into remission, which means that a person is free of any signs of the disease for varying periods of time.
In only about 10% of cases, the cancer cells spread to the organs of the body or the blood. Today, most patients diagnosed in stages one and two can gain treatments which will control the cancer.
A study of survival data obtained from Stanford University School of Medicine showed that patients with T1 disease who were treated had a median survival of 32.5 years.
