Mycosis fungoides (MF), also known as malignant cutaneous reticulosis and granuloma fungoides, is a rare, chronic malignant T-cell lymphoma of unknown cause that originates in the reticuloendothelial system of the skin, eventually affecting lymph nodes and internal organs. Unlike other lymphomas, MF allows an average life expectancy of 7 to 10 years after diagnosis. If correctly treated, particularly before it has spread beyond the skin, MF may go into remission for many years. However, after MF has reached the tumor stage, progression to severe disability or death is rapid.
The cause of MF is unknown. Most persons with MF have it for years and it can lead to death, but this is unusual.
In the United States, MF strikes more than 1,000 people of all races annually; most are between ages 40 and 60.
The first sign of MF may be generalized erythroderma, possibly associated with itching. Eventually, MF evolves into varied combinations of infiltrated, thickened, or scaly patches, tumors, or ulcerations.
Other diagnostic tests help confirm MF: complete blood count and differential; a finger-stick smear for Sézary cells (abnormal circulating lymphocytes), which may be present in the erythrodermic variants of MF (Sézary syndrome); blood chemistry studies to screen for visceral dysfunction; chest X-ray; liver-spleen isotopic scanning; lymphangiography; and lymph node biopsy to assess lymph node involvement. These tests also help to stage the disease — a necessary prerequisite to treatment.
Depending on the stage of the disease and its rate of progression, past treatment and results, the patient's age and overall clinical status, treatment facilities available, and other factors, treatment of MF may include topical, intralesional, or systemic corticosteroid therapy; phototherapy; methoxsalen photochemotherapy; radiation; topical, intralesional, or systemic treatment with mechlorethamine (nitrogen mustard); and other systemic chemotherapy.
Application of topical nitrogen mustard is the preferred treatment for inducing remission in pretumorous stages. Plaques may also be treated with sunlight and topical steroids.
Total body electron beam radiation, which is less toxic to internal organs than standard photon beam radiation, has induced remission in some patients with early stage MF.
Chemotherapy is employed primarily for patients with advanced MF; systemic treatment with chemotherapeutic agents (cyclophosphamide, methotrexate, doxorubicin, bleomycin, etoposide, and steroids) and interferon-alfa produces transient regression.
❑If the patient has difficulty applying nitrogen mustard to all involved skin surfaces, provide assistance. However, wear gloves to prevent contact sensitization and to protect yourself from exposure to chemotherapeutic agents.
❑If the patient is receiving drug treatment, report adverse effects and infection at once.
❑The patient who's receiving radiation therapy will probably develop alopecia and erythema. Suggest that he wear a wig to improve his self-image and protect his scalp until hair regrowth begins, and suggest or give medicated oil baths to ease erythema.
❑Because pruritus is generally worse at night, the patient may need larger bedtime doses of antipruritics or sedatives, as ordered, to ensure adequate sleep. When the patient's symptoms have interrupted sleep, postpone early morning care to allow him more sleep.
❑The patient with intense pruritus has an overwhelming need to scratch — in many cases to the point of removing epidermis and replacing pruritus with pain, which some patients find easier to endure. Realize that you can't keep such a patient from scratching; the best you can do is help minimize the damage. Advise the patient to keep fingernails short and clean and to wear a pair of soft, white cotton gloves when itching is unbearable.
❑The malignant skin lesions are likely to make the patient depressed, fearful, and self-conscious. Fully explain the disease and its stages to help the patient and family understand and accept the disease. Provide reassurance and support by demonstrating a positive but realistic attitude. Reinforce your verbal support by touching the patient without any hint of anxiety or distaste.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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Title: Professional Guide to Diseases (Eighth Edition)
Authors: Springhouse
Publisher: Lippincott Williams & Wilkins
Copyright: 2005
ISBN: 1-58255-370-X
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