Provincial Health Services Authority (PHSA) improves the health of British Columbians by seeking province-wide solutions to specialized health care needs in collaboration with BC health authorities and other partners.
Revised 7 June 2010
Patients with CTCL including mycosis fungoides or Sézary syndrome can be referred to the Skin Lymphoma Group at the BC Cancer Agency, Vancouver Centre to arrange for appropriate treatment or for management opinion only. The Skin Lymphoma Group consists of representatives from Dermatology, Radiation Oncology, Medical Oncology and Pathology. Referrals can be made through the Admitting Department of the Vancouver Centre.
Patients with biopsy-confirmed CTCL are staged according to the extent of their skin disease and treatment is determined by their stage. The staging system used is as follows:
No skin involvement
Patches or plaques, covering<10% of the body surface
Same as above but covering >10% of the body surface
Tumors with or without patches or plaques
No abnormal peripheral lymph nodes
Abnormal peripheral lymph nodes, biopsy negative
(i.e., dermatopathic lymphadenopathy)
Abnormal peripheral lymph nodes, biopsy positive
No abnormal visceral lymph nodes
Abnormal visceral lymph nodes (no information regarding biopsy)
Abnormal visceral lymph nodes, biopsy negative
Abnormal visceral lymph nodes, biopsy positive
No visceral organ involvement
Visceral organ involvement (on basis of histology)
<5% circulating Sézary cells and <250 Sézary cells per cc
>5% circulating Sézary cells, or>250 Sézary cells per cc
Prior to staging, each patient has a careful clinical examination. If any superficial lymph nodes are enlarged, and the disease is otherwise clinically confined to the skin, the largest node is biopsied. A CT scan of the abdomen and pelvis is done to assess the intra-abdominal and pelvic lymph nodes. If an abnormal node is demonstrated, (usually in the pelvis) and the disease is clinically confined to the skin, biopsy is considered. A quantitative Sézary cell count is performed to assess the presence and number of circulating lymphoma cells as well as a CBC and LDH.
Large plaque parapsoriasis (poikiloderma vasculare atrophicans) can progress to mycosis fungoides. Treatment of large plaque parapsoriasis includes UVB or PUVA therapy. Patients with large plaque parapsoriasis should be followed at regular intervals (at least every six months). Sequential biopsies of the thickest lesions may be necessary to confirm progression to a true Cutaneous T cell lymphoma such as mycosis fungoides or Sézary syndrome.
Follicular mucinosis or lesions similar to lymphomatoid papulosis may be associated with mycosis fungoides in 10-15% of cases. Assessment at the Vancouver Cancer Centre may be useful. True lymphomatoid papulosis is a separate disease and does not evolve into T-cell lymphoma (see section on Lymphoma, special problems).
CTCL includes a number of clinical syndromes, the most common of which are mycosis fungoides and the Sézary syndrome. Mycosis fungoides presents with patches, plaques, or tumors. In the initial stages disease is confined to the skin. Sézary syndrome is a leukemic variant of CTCL, characterized by erythroderma (stage T4), lymphadenopathy, pruritus and circulating neoplastic (Sézary) cells (stage B1).
a) Patch or Plaque stage disease limited to geographical region that can be reasonably encompassed by limited radiation field(s)b) Diffuse patch or plaque stage disease: Patients with diffuse involvement of the skin are treated with topical therapy. There is no evidence that one topical therapy is superior in terms of efficacy or side effects. Treatment is continued in an adjuvant fashion until 1 year AFTER clearance of clinical disease Skin Directed therapies include:
a) Advanced / refractory cutaneous disease : Occasionally if disease is refractory to one of these topical therapies, multi-agent therapy may be recommended. This may include:
However, after exhaustion of above mentioned therapies, with progressive disease, systemic chemotherapy can be used (see below).b) Systemic disease: occasionally patients present with systemic disease and will need to be on systemic chemotherapy. There is no clear evidence that one medication is superior than another in terms of efficacy. The usual order of use is:
c) Special circumstances:
occasionally > 5% circulating tumour cells/ sezary cells are seen. These patients are usually treated as above. However, for patients with refractory disease, who have circulating tumour cells, Extra-Corporeal Photoimmunotherapy has been used. (See chemo protocol ULYMFECP)
Reference: EORTC consensus recommendations for the treatment of mycosis Fungoides/ Sezary Syndrome EJC 42(2006) p1014-1030
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