Cutaneous T cell lymphoma (CTCL) is a term that applies to T cell lymphoma first manifested in the skin, but since the neoplastic process involves the entire lymphoreticular system, the lymph nodes and internal organs become involved in the course of the disease. CTCL is a malignancy of helper T cells (CD4+).
Causes of Cutaneous T Cell Lymphoma
CTCL is a rare disease – five to ten persons per million are affected. The cause of CTCL remains unknown, but research continues. CTCL is not contagious and is not inherited. Men are affected more than women, and it is more common after the age of 50.
Symptoms of Cutaneous T Cell Lymphoma
The symptoms of Cutaneous T Cell Lymphoma are seen primarily in the skin, with itchy red patches or plaques and, usually over time, mushroom-shaped skin tumors. Any part of the skin can be involved and the extent and distribution of the rash or tumors vary greatly from patient to patient. The only really universal symptom of the disease is the itch and this symptom is usually what brings the patient to the doctor for treatment. If the disease spreads outside of the skin, the symptoms include swelling of the lymph nodes, usually most severe in those draining the areas with skin involvement
Diagnosis
In the early stages, the diagnosis of CTCL is a problem. Clinical lesions may be typical, but histologic confirmation may not be possible for years despite repeated biopsies. One-micrometer thick sections may be helpful. For early diagnosis, cytophotometry (estimation of aneuploidy and polyploidy) and estimation of the indentation of pathologic cells (nucleocontour index) are helpful. Fresh tissue should be sent for immunophenotyping of infiltrating T cells by use of monoclonal antibodies and T cell receptor rearrangement studies. Lymphadenopathy and the detection of abnormal circulating T cells in the blood appear to correlate well with internal organ involvement. See TNM classification and staging.
Treatment
In the pre-CTCL stage, in which the histologic diagnosis is only compatible, but not confirmed, PUVA photochemotherapy is the most effective treatment. For histologically proven plaquestage disease with no lymphadenopathy and no abnormal circulating T cells, PUVA photochemotherapy is also the method of choice. Also used at this stage are topical chemotherapy with nitrogen mustard in an ointment base (10 mg/dL) and total body electron-beam therapy, singly or in combination. Isolated tumors that may develop should be treated with local x-ray or electron-beam therapy. For extensive plaque stage with multiple tumors or in patients with lymphadenopathy or abnormal circulating T cells, electron-beam plus chemotherapy is probably the best combination for now; randomized, controlled studies of various combinations are in progress. Also, extracorporeal PUVA photochemotherapy is being evaluated in patients with Sezary’s syndrome.
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