Cutaneous T- cell lymphoma (CTCL)

Lymphomas are generally carcinomas of the lymphatic system; however, lymphomas can occur in the skin with no evidence of disease elsewhere. They are referred to as primary T-cell lymphomas and account for 65% of lymphoma’s affecting the skin. Cutaneous T-cell lymphomas (CTCL) refer to a serious but uncommon skin condition in which there is an abnormal neoplastic proliferation of lymphocytes with a ‘T’ subtype (thymus derived).

Mycosis fungoides
Mycosis fungoides is the most common type of CTCL presenting as patches or lumps composed of white cells called lymphocytes. It generally follows a low-grade clinical course, often persisting slowly over years in a patch stage, then slowly progressing to the tumour stage (DermNet NZ, 2009b) (see Less common skin conditions for further detail).

Primary cutaneous CD30+ lymphoproliferative disorders
This is the second most common group of CTCL and accounts for about 30% of all CTCL cases. This group includes primary cutaneous anaplastic large cell lymphoma, which presents as solitary or localised nodules or tumours. Prognosis is usually good but there may be regional lymph node involvement for 10% of patients. The other common type in this group is lymphomatoid papulosis, with lesions occurring predominantly on the trunk and limbs; they clear spontaneously but may scar (DermNet NZ, 2009b).

Sézary syndrome
Sézary syndrome (sometimes referred to as ‘red man syndrome’) is the name given when T-cell lymphoma affects the skin of the entire body. The skin is also thickened, dry or scaly and usually very itchy. Examination usually reveals the presence of neoplastic T cells (Sézary cells) in skin, lymph nodes and peripheral blood. The prognosis of Sézary syndrome is generally poor with a median survival between 2 and 4 years; infection caused by immunosuppression is the main cause of death (DermNet NZ, 2009b).

Treatment of CTCL
Treatments of CTCL depend on the tumour, site, stage, distribution, age and general health of the patient. Various treatments may include topical steroids, phototherapy, photophoresis, topical nitrogen mustard, chemotherapy, radiotherapy, interferons and oral retinoids.