Cutaneous T-Cell Lymphoma (CTCL)

What is cutaneous T-cell lymphoma (CTCL)?

Lymphoma” is the name for types of cancer that arise in the lymphocytes (immune cells). The three different types of lymphocytes include B-cells, T-cells, and natural killer (NK) cells. T lymphocytes have many functions, including helping B cells and NK cells fight viruses and cancer cells. Lymphoma occurs most commonly in B cells, but in the skin T cell lymphomas are the most common type. Lymphoma is divided in two categories: Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL). Cutaneous T-cell lymphoma is a rare type of NHL that occurs primarily in the skin and has a range of signs, symptoms, and treatment options. There are two main subtypes of CTCL: 
 
  • Mycosis fungoides (MF): most common form of CTCL. Generally affects the skin, but may progress internally. Symptoms include rash, tumors, skin lesions, and itchy skin. Most cases occur in people over the age of 20, and it occurs more commonly in men.
  • Sézary syndrome (SS): an advanced form of MF, in which lymphoma cells are present in the blood. Thin, red, itchy rashes usually cover over 80% of the body. Patches and tumors may appear in certain patients. Changes may occur in the nail, hair, eyelids, or lymph nodes.  
 
 
Risk factors
The actual causes of CTCL remain unknown, but expert theories have linked the condition to viral infection, chemical exposure, and genetic changes. The following factors may increase one's chance of developing the disease:
 
  • Gender. CTCL is twice as common in men than in women.
  • Race. CTCL is more common in blacks than in whites.
  • Older age. Incidence goes up as age increases.
 
 
Diagnosis of CTCL can take years, since early on it mimics other skin disorders such as eczema and psoriasis. Diagonostic procedures usually include:
 
  • Incisional skin biopsy: a small portion of a skin lesion is removed and inspected under a microscope to determine if the cells indicate CTCL.
  • Full physical exam: including palpation of lymph nodes and blood work to check for cancerous lymphocytes.
  • Computerized tomography (CT) scan, positron emission tomography (PET) scan, or magnetic resonance imaging (MRI) to check if cancer has spread to lymph nodes and organs.
 
 
Staging 
The extent of MF and SS are determined using the following staging system:
 
  • Stage I
  • Stage IA: less than 10% of skin surface covered with patches and/or plaques.
  • Stage IB: 10% or more of skin surface covered in patches and/or plaques.
  • Stage II
  • Stage IIA: any amount of skin surface covered in patches and/or plaques. Lymph nodes are enlarged but do not contain cancerous cells. 
  • Stage IIB: one or more tumor lesions found on the skin. Lymph nodes may be enlarged, but do not contain cancerous cells.
  • Stage III
Greater than 80% of the body surface is covered with red patches or plaques. Lymph nodes may be enlarged but do not contain cancerous cells. 
 
  • Stage IV
  • Stage IVA: any amount of skin surface is covered with patches, plaques, or tumors. Cancer involves lymph nodes and/or blood.
  • Stage IVB: Same criteria as IVA, but cancer also involves other organs in the body.
 
 
Treatment for CTCL depends on the extent of the affected skin, the type of skin lesion, and whether the cancer has spread to lymph nodes or other organs in the body. MF treatment is either directed at the skin alone, or the entire body (systemic). Since SS is usually chronic and affects the whole body, skin-directed therapies alone are usually not enough. More advanced stages of the disease usually involve  chemotherapy, radiation, and other forms of therapy such as allogeneic stem cell transplantation (high risk disease patients).
 
 
 
 
# CUTANEOUS T-CELL LYMPHOMA (CTCL) BY THE NUMBERS #
  • The overall incidence of CTCL is approximately six cases per one million individuals
  • In the US, there are approximately 1,500 new CTCL cases per year
  • Cutaneous T-cell lymphomas account for about 4% of all cases of non-Hodgkin lymphomas
  • MF and SS are usually diagnosed in people between ages 50 and 60
  • CTCL is twice as common in men as it is in women
 
about cutaneous t-cell lymphoma (CTCL)
 
 

Sources
  • "Cutaneous T-Cell Lymphoma (CTCL)." Cutaneous T-Cell Lymphoma - Lymphoma Research Foundation. N.p., n.d. Web. 10 July 2017.
  • Gknation. "Treatment for Indolent NHL Subtypes." Gknation. N.p., 26 Feb. 2015. Web. 10 July 2017.
  • Cancer Resources from OncoLink | Treatment, Research, Coping, Clinical Trials, Prevention. "All About Cutaneous T Cell Lymphoma (CTCL)." OncoLink. N.p., n.d. Web. 10 July 2017.
  • "DermNet New Zealand." Cutaneous T-cell Lymphoma | DermNet New Zealand. N.p., n.d. Web. 10 July 2017.
  • "Cutaneous Lymphoma." Columbia University Medical Center. N.p., n.d. Web. 10 July 2017.

 

Cutaneous T-Cell Lymphoma (CTCL) is a rare type of cancer that begins in the white blood cells, called T-lymphocytes (T-cells), and attacks the skin. T-cells are important to the immune system and help the body fight infection(s). However, with an abnormal and rapid production of these cells, T-cells can accumulate in the body and cause cancer. CTCL also can involve the blood, the lymph nodes, and other internal organs. Check out this infographic to learn more about CTCL:
 
cutaneous t-cell lymphoma

 

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