Cutaneous T-cell Lymphoma
Cutaneous T-cell lymphomas, known as CTCLs, are serious but rare disorders. Lymphomas are cancers of the lymphatic system, a part of the immune system that produces white blood cells and helps fight infections and diseases. Lymphomas account for fewer than 5 percent of all cancers and a small percentage of non-Hodgkin's lymphomas are CTCLs.
Causes of CTCL
CTCLs are disorders that result from malignant T-cells that multiply uncontrollably, traveling to the skin. The cause of CTCL is unknown although there is some evidence that patients who develop CTCL have had a pre-existing allergic dermatitis or have been infected with a retrovirus. CTCL is more common in men than in women and more frequently occurs in middle-aged men of African descent. There is some evidence to suggest that environmental exposure to certain chemicals, particularly through occupational hazards, may increase its prevalence.
Types of CTCL
CTCLs are defined as either low-grade lymphomas, known as indolent, or high-grade lymphomas, known as aggressive. Most CTCLs are in the former category, developing very slowly. The two most common types of CTCL are mycosis fungoides and Sézary's syndrome. It is unclear at this time whether the latter is a progressive form of the former or whether these are two different types of the illness.
Mycosis Fungoides
This variety of CTCL is by far the most common of the CTCLs, accounting for nearly three-quarters of the cases of this disease. While the illness may remain in its early stages for several years, it may also progress through its four identified stages, moving from plaques on the skin to ulcerating, infected, or even necrotic, tumors. While the name of this disorder seems to imply a connection to fungal infections, there is no such connection.
Sézary's Syndrome
About 5 percent of the cases of mycosis fungoides may appear later as cases of Sézary's syndrome, a more serious disorder in which the diseased leukocytes become grossly abnormal in shape. These blood cells, known as Sézary's cells, spread into the lymph nodes and bloodstream, becoming more lethal.
Symptoms of CTCL
In CTCL's early stages, red patches or plaques appear on the skin. At first, these patches may resemble the rashes of eczema, contact dermatitis, or psoriasis. In addition to the red rash, patients may experience dry skin, severe itching and enlarged lymph nodes.
Diagnosis of CTCL
There are many methods use to diagnose CTCL which include the following:
- Complete blood count, or CBC
- Peripheral blood smear
- Tissue biopsy
- Microscopic cellular analysis
Treatment of CTCL
There are several effective treatments for CTCL. It is important to diagnose the condition as early as possible so treatment can be administered before the disease progresses. Treatments may include:
- Skin creams
- Light therapy
- Medications, at times including interferon
- Radiation therapy
- Chemotherapy
- Photopheresis, an extracorporeal blood treatment
Recovery from CTCL
It is difficult to cure CTCL. Most of the treatment options offer palliative care to relieve symptoms and improve quality of life. While the prognosis depends on the stage of the disease and the nature of the lesions, many patients may live for many years with CTCL.