Mycosis Fungoides and Sézary Syndrome

The following are symptoms of cutaneous T-cell lymphoma:

• Round skin patches that may be raised or scaly and itchy
• Distinct patches on the skin
• Developing skin lumps that could rupture
• Increased lymph nodes
• Losing hair
• The skin on the palms and soles gets thicker
• A rash-like, extremely irritating redness of the skin that covers the entire body

The stages of mycosis fungoides and Sezary syndrome are:

1. Stage I (Early mycosis fungoides)
   • In the blood, very few Sezary cells may be present.
     1. Stage I A: Less than 10 percent of the skin's surface has patches, papules, or plaques.
     2. Stage IB: Patches, papules, and/or plaques encircle 10 percent or more of the skin's surface.
2. Stage II (Mycosis fungoides)
   • Some Sezary cells may exist in the blood.
     1. Stage IIA: Patches, papules, and/or plaques cover any exposed skin surface. Lymph nodes are abnormal but do not have malignancy.
     2. Stage IIB: There are one or more skin malignancies, about 1 cm big. Lymph nodes could be abnormal, but they are not cancerous.
3. Stage III mycosis fungoides
   • The skin is reddish on 80 percent or more of its surface and may feature tumors, papules, plaques, or patches. While aberrant, lymph nodes are not malignant.
   • There might be a few Sezary cells present in the blood.
4. Stage IV (mycosis fungoides or Sezary syndrome)
   • A high number of Sezary cells are present in the blood.
     1. Stage IVAi: Any portion of the skin surface could be covered in patches, papules, plaques, or tumors; 80 percent or more of the skin surface could be reddish. Although the lymph nodes could be aberrant, they are not cancerous. The blood contains many Sezary cells.
     2. Stage IVAii: 80 percent or more of the skin could be affected. Either cancer has developed in the lymph nodes, or the lymph nodes are highly aberrant. The blood may include a significant amount of Sezary cells.
     3. Stage IVB: Cancer has spread to the liver or spleen, among other internal organs. Any portion of the skin could be affected. The lymph nodes could be malignant or abnormal. The blood may include a significant amount of Sezary cells.

There are some standard therapies for mycosis fungoides and Sezary syndrome; others are undergoing clinical trials. A clinical trial is a research project designed to learn more about potential new cancer treatments or refine existing ones. A new treatment might replace the existing one if clinical studies reveal that it is superior to the current one.

Your healthcare provider will recommend treatments based on the stage of the disease and its symptoms.

Treatment options include:

• Radiation therapy: Eradicates cancer cells or stops them from proliferating using high-energy X-rays or other forms of radiation. As part of external radiation therapy, equipment placed outside the body emits radiation in the direction of the cancerous area. Mycosis fungoides and Sezary syndrome are occasionally treated using total skin electron beam radiation therapy. A radiation therapy machine directs electrons at the skin that covers the entire body. The palliative application of external radiation treatment is to treat symptoms and enhance the quality of life. Ultraviolet A or B radiation therapy can be applied using a particular lamp or laser that emits radiation to the skin.
• Chemotherapy: Employs medications to kill or prevent the division of cancer cells. Chemotherapy medications can reach cancer cells throughout the body when administered orally, intravenously, or intramuscularly. Sometimes, chemotherapy is used topically (applied to the skin in the form of a cream, lotion, or ointment).
• Photodynamic therapy: Employs a drug and a particular type of laser light. The medication is administered intravenously. Cancer cells accumulate the drug more than healthy cells do. When a laser beam is shone onto the skin to treat skin cancer, the drug activates and begins to kill the cancer cells. Photodynamic therapy only mildly damages healthy tissue. People undergoing photodynamic therapy must limit their time outside in the sun.
• Drug therapies: Topical corticosteroids (class I compounds) are an effective treatment for patch-stage mycosis fungoides and Sezary syndrome. Cancer cell growth can be slowed by using retinoid creams or gels. Histone deacetylase inhibitors slow down and stop tumor cell division.
• Immunotherapy: The body's natural defenses against cancer are boosted, directed, or restored using substances produced by the body or in a lab. Biologic therapy is a category of treatment for cancer.
• Interferon: Slows the growth of tumors by interfering with the division of Sezary and mycosis fungoides cells.
• Targeted therapy: A type of medical care that isolates and destroys cancer cells using medicines or other substances. Compared to chemo or radiation therapy, targeted therapies typically cause less damage to normal cells.