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Mycosis Fungoides

 
General Information / Anatomy / Function / Statistics
Very rare type of lymphoma that is usually discussed separately because it behaves so differently from other lymphomas
Slow growing.
Affects the skin where it almost always starts.
Remains on the skin for years before spreading to other parts of the body.
Very difficult to diagnose because symptoms are similar to other less serious skin diseases.
Only in advanced cases are there enough lymphoma cells concentrated on the skin to make a confirmed diagnosis by biopsy.
For this reason, even though symptoms may have been noted for years and the patient may have been under the care of a dermatologist (skin specialist), it is not usually diagnosed in people under 40.
Fifteen cases each year expected in BC
Patients with stage I or II usually have a very long life expectancy and a proportion will never relapse after treatment
Nothing to do with a fungus infection

Symptoms / Signs
  • chronic intermittent, itchy rash that can appear anywhere on the surface of the body
  • In early stages it appears as patches of thick, scaly skin (called plaques) that look like eczema or psoriasis

Diagnosis / Staging / Grading / Types
  • Diagnosis can only be made on the basis of biopsy examination under a microscope of affected skin cells removed by minor surgery in the doctor's office
  • To determine the extent of the disease the following procedures may be requested:
    • Immunological laboratory tests on blood
    • Chest X-ray
    • CT scan of the abdomen
    • Examination of the blood for lymphoma cells and other blood tests

Treatment
Depends on stage of disease
The premycotic stage Produces the symptoms described above. However it is impossible to get a confirmed diagnosis. Treatment focuses on relieving the itching with soothing baths, anti-histamines, and creams containing steroid hormones.
Stage I The disease is confined to the skin, forming thin patches or thicker plaques (where the lymphoma cells have spread into the lower levels of the skin).
Stage II Either as above together with enlarged lymph nodes which are not found to contain lymphoma cells if biopsied , or forming a solid tumour or lump in the skin.
Stage III Widespread redness of the skin (erythroderma).
Stage IV Any of the above together with either lymph nodes which on biopsy are found to contain lymphoma cells or the presence of lymphoma cells in the blood or internal organs or bone marrow.
Treatment of stages I and II may be with either a chemotherapy agent applied to the skin or with ultraviolet light therapy together with a drug to sensitize the lymphoma cells to the light. Radiotherapy may be used for stubborn areas which do not respond to the other treatments. Radiation therapy is usually very effective. Radiotherapy to the whole skin surface may be used if these treatments are not effective.
Stages III and IV are usually treated with chemotherapy. Whole skin radiotherapy is not used here. Radiotherapy is also usually very effective for isolated stubborn areas or for lumps (tumours) which are causing problems.

Revised November 2000

 


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