Rare Classroom: Cutaneous T-Cell Lymphoma

Welcome to the Rare Classroom, a new series from Patient Worthy. Rare Classroom is designed for the curious reader who wants to get informed on some of the rarest, most mysterious diseases and conditions. There are thousands of rare diseases out there, but only a very small number of them have viable treatments and regularly make the news. This series is an opportunity to learn the basics about some of the diseases that almost no one hears much about or that we otherwise haven’t been able to report on very often.

Eyes front and ears open. Class is now in session.

The disease that we will be learning about today is:

Cutaneous T-Cell Lymphoma 

What is Cutaneous T-Cell Lymphoma?

  • Cutaneous T-cell lymphomas (CTCLs) constitute a group of non-Hodgkin lymphomas (NHLs) of the skin.​
  • Mycosis Fungoides (MF) is the most common form of CTCL. Because of that, the terms MF and CTCL are often used interchangeably, and sometimes imprecisely. This can be a source of confusion. All cases of MF are CTCL, but not all CTCL cases are MF​
  • In this group of cancers, the affected T-cells migrate to the skin, causing the appearance of lesions.

How Do You Get It?

  • CTCL is twice as common in men as in women.
  • CTCL is more common in African-Americans.​
  • The overall annual age-adjusted incidence of CTCL is approximately six cases per one million.​
  • While the number of new cases diagnosed each year is relatively low (about 3,000), it is estimated that, since patients have a very long survival, there may be as many as 30,000 patients living with cutaneous lymphoma in the United States and Canada.​
  • Due to the difficulty of diagnosing the disease in its early stages and the lack of an accurate reporting system, these numbers are estimates.​
  • Although there is continuing research, at this time no single factor has been proven to cause this disease. There is no supportive research indicating that it is genetic or hereditary. Studies have failed to show connections between chemical exposure, environment, pesticides, radiation, allergies and occupations.
  • Exposure to Agent Orange may be a risk factor for developing CTCL for veterans of the Vietnam War, but no direct cause-effect relationship has been established. ​​

What Are The Symptoms?

  • Though the appearance and pattern of skin lesions differ, the various forms of cutaneous T-cell lymphoma are characterized by some form of rash or skin abnormality.
    • In mycosis fungoides, the most common type, the skin first presents with a scaly, red rash that appears on areas of skin not exposed to the sun. This eventually progresses to raised papules or lesions and eventually tumors that appear on the skin. In Sézary syndrome, the other most common type, a bright red rash can cover much of the body.
  • Other common symptoms include:
    • Dermatitis (generalized skin inflammation)
    • Enlarged lymph nodes
    • Enlarged liver and spleen

How Is It Treated?

  • Cutaneous T-cell lymphoma cannot be cured
  • However, there are a number of treatment options available.
  • Though in some cases symptoms can cause serious pain and debilitation, some patients can live mostly normal lives with effective treatment.
  • FDA approved treatments for this group of cancers include:
    • Denileukin diftitox
    • Bexarotene
    • Vorinostat
    • Romidepsin
    • Poteligeo
  • A number of therapies are also employed in an off-label capacity:
    • Forodesine
    • Methotrexate
    • Corticosteroids
    • Cyclophosphamide
    • Gemcitabine
    • Pentostatin
    • Carmustine
    • Liposomal doxorubicin
    • Carmustine
    • Mechlorethamine
    • Stem cell transplant
    • Phototherapy
    • Alemtuzumab
    • Interferons
    • Skin electron therapy
    • Photopheresis

Where Can I Learn More???

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