Cutaneous T cell Lymphoma

What is Cutaneous T cell Lymphoma?

Lymphoma (cancer in the lymphatic system) can be divided into two broad categories: Hodgkin's and non-Hodgkin's. Cutaneous T cell lymphoma (CTCL) is a type of non-hodgkin's lymphoma. Cutaneous (which means of the skin) T cell lymphoma includes a wide variety of lymphomas that have a common feature: the cancerous white blood cells are confined to the skin (localised) when the disease is diagnosed. The lymphocytes (white blood cells produced by the lymphatic system) involved in CTCL are a special group of lymphocytes called T cells that maintain the normal function of the skin immune system. There are various forms of cutaneous T cell lymphoma , and there continues to be confusion in the classification. The most common forms of cutaneous T cell lymphoma are mycosis fungoides (MF) and Sezary syndrome (SS), which make up the majority of this disease. MF predominantly involves the skin, whereas in SS there is also blood involvement.

Statistics on Cutaneous T cell Lymphoma?

Cutaneous T cell lymphoma constitutes about 3% of all lymphomas.Mycosis fungoides/Sezary syndrome is the most common form of cutaneous T cell lymphoma. Cutaneous T cell lymphoma more common in males and in blacks. About half of the patients are in their mid-fifties when the cutaneous T cell lymphoma starts.

Risk Factors for Cutaneous T cell Lymphoma

The aetiology is unknown.Several possible causes have been proposed:1. Bacterial infection of the skin.2. Long-term exposure to chemicals.3. Smoking.4. Medications.5. Long-term sun exposure.6. Viral infections.

Progression of Cutaneous T cell Lymphoma

Mycosis fungoides/Sezary syndrome is a disease that develops in a slow and subtle way. In the early phase of the disease, patients may have only skin abnormalities and the disease may not be picked up for years. Cutaneous T cell lymphoma may wax and wane for the early phase, making it difficult to diagnose with certainty.Cutaneous T cell lymphoma mainly involves the skin and lymph nodes, and later, other organs. Staging of cutaneous T cell lymphoma is from Stage I to IV:

Stage I: when cutaneous T cell lymphoma is limited to the skin, without lymph node or organ involvement.

Stage II: when there is a peripheral lymph node which appears abnormal on clinical examination, or when the skin disease has already reached the cancerous stage.

Stage III: when there is generalised skin reddening (erythroderma).

Stage IV: when biopsy (taking out samples from body structures) reveals features of CTCL or when the disease has spread to other organs.

How is Cutaneous T cell Lymphoma Diagnosed?

A sample will be taken from the skin to be looked under the microscope for diagnosis.Blood will also be drawn to check for the number of white blood cells and malignant cells circulating in the blood.

Prognosis of Cutaneous T cell Lymphoma

Most cutaneous T cell lymphoma patients at Stage I survive for more than 12 years.

At Stage II and III, most cutaneous T cell lymphoma patients survive for 5 years.

At Stage IV, most cutaneous T cell lymphoma patients survive for 2.5 years.Some cutaneous T cell lymphoma patients (about 8% - 23%) may undergo changes in the disease causing it to change to another type of lymphoma - large cell lymphoma. This type of patients has a poorer outcome, with an average survival ranging from 2 to 19 months.

How is Cutaneous T cell Lymphoma Treated?

Cutaneous T cell lymphoma patients at an early stage can be treated with radiotherapy, which in this case if often a type called total-skin electron beam irradiation.For more advanced disease, cutaneous T cell lymphoma treatment is often to relieve symptoms or pain. Options include topical glucocorticoids (steroids applied on the skin), phototherapy (light therapy), psoralen with ultraviolet A (PUVA) (another form of light therapy), electron beam radiation, antibodies, and chemotherapy.

Cutaneous T cell Lymphoma References

[1] Detailed Guide: Lymphoma, Non-Hodgkin's type. What Is Non-Hodgkin Lymphoma? [online]. 2004 [cited 2005 July 29]. Available from: URL: http://www.cancer.org/docroot/cri/content/cri_2_4_1x_what_is_non_hodgkins_lymphoma_32.asp[2] Kasper D et al. Harrison's principles of internal medicine. 16th ed. New York; McGraw-Hill; 2005.[3] Siegel RS, Pandolfino T, Guitart J, et al: Primary cutaneous T-cell lymphoma: review and current concepts. J Clin Oncol 2000 Aug; 18(15): 2908-25.