In the United States, there are about 1,500 new cases of cutaneous T-cell lymphoma per year, according to the Leukemia & Lymphoma Society. Men are twice as likely as women to be affected, and most individuals are diagnosed after age 50. Cutaneous T-cell lymphoma does not usually affect children. It is not known what causes this type of cancer to develop.
Types of cutaneous T-cell lymphoma
Several different disorders are considered to be cutaneous T-cell lymphomas. The most common is mycosis fungoides (44% of cases) which includes the subtypes:- Sezary syndrome (advanced form of mycosis fungoides) - 3%
- Folliculotropic mycosis fungoides - 4%
- Pagetoid reticulosis (Woringer-Kolopp disease) - <1%
- Granulomatous slack skin - <1%
- Primary cutaneous CD30+ anaplastic large cell lymphoma - 8%
- Lymphomatoid papulosis - 12%
- Subcutaneous panniculitis-like T-cell lymphoma - 1%
- Primary cutaneous CD4+ T-cell lymphoma - 2%
- Primary cutaneous peripheral T-cell lymphoma - 2%
Symptoms
In most types of CTCL, the symptoms begin as flat, red patches on the skin; in dark-skinned individuals these may appear as either very light or very dark patches. The patches are very itchy, and may be dry and scaly. Some areas of skin may become raised and hard (called plaques). Later on tumors may develop. Some skin folds become thickened and crack, leading to infection. The skin tumors may also become infected.Like other cancers, as CTCL continues it begins to move to other parts of the body. The first parts affected are the lymph nodes, which become inflamed, and often cancerous. Cancer may spread to the liver, lungs, or bone marrow.
Diagnosis
The skin symptoms that are present may suggest a diagnosis of CTCL. However, since many other skin disorders cause similar symptoms, a skin sample (biopsy) is used to look for abnormal cells. Examination of blood cells may reveal skin lymphoma cells in the blood. Inflamed lymph nodes can be examined for cancer cells, and computed tomography (CT) scan and magnetic resonance imaging (MRI) can look for cancer elsewhere in the body.Treatment
Treatment for cutaneous T-cell lymphoma depends on the type and how far it has spread. If the cancer is confined to the skin, it may be treated with chemotherapy such as Mustargen (nitrogen mustard) applied to the skin. Another treatment is the drug psoralen combined with ultraviolet-A light. High-dose Roferon-A (interferon alfa-2a) may also be given. Electron beam radiation can be applied to the skin surface without affecting internal organs. Skin-softening and anti-itch creams can help control symptoms.
If the cancer has spread, chemotherapy such as Cytoxan (cyclophosphamide), Adriamycin (doxorubicin), Oncovin (vincristine), and prednisone may be used.
Orphan drug treatments
Several orphan drugs have become available in recent years to treat CTCL. They are:- Targretin (bexarotene) for early stage CTCL, hard to treat cutaneous lymphoma, and in combination with other treatments (FDA approved December 1999)
- Ontak (denileukin diftitox) for advanced CTCL, or CTCL that comes back after treatment (FDA approved February 1999)
- Zolinza (vorinostat) for hard to treat or advanced CTCL, or CTCL that comes back after treatment (FDA approved October 2006)
Sources:
FS-5 - Cutaneous T Cell Lymphoma. 25 Oct 2004. Leukemia & Lymphoma Society, accessed 2 Nov 2006.
Roberts, Scott. "Zolinza Treats Rare Skin Cancer." MSN Health & Fitness 06 Oct 2006, accessed 02 Nov 2006.
