Revised: 30 May 2003

• Staging
• Management
  • 3.1 Pre-lymphoma
  • 3.2 Cutaneous T Cell Lymphoma (CTCL)
  • 3.3 Limited Disease
  • 3.4 Advanced Disease
  • 3.5 Relapsed Disease after TBE, Clinically Limited to the Skin

1 Introduction

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.

2 Staging

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:

T0	No skin involvement
T1	Patches or plaques, covering <10% of the body surface
T2	Same as above but covering >10% of the body surface
T3	Tumors with or without patches or plaques
T4	Generalized erythema
NP0	No abnormal peripheral lymph nodes
NP1-	Abnormal peripheral lymph nodes, biopsy negative
	(i.e., dermatopathic lymphadenopathy)
NP1+	Abnormal peripheral lymph nodes, biopsy positive
NV0	No abnormal visceral lymph nodes
NV1	Abnormal visceral lymph nodes (no information regarding biopsy)
NV1-	Abnormal visceral lymph nodes, biopsy negative
NV1+	Abnormal visceral lymph nodes, biopsy positive
M0	No visceral organ involvement
M1	Visceral organ involvement (on basis of histology)
B0	<5% circulating Sézary cells and <250 Sézary cells per cc
B1	>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.

3. Management

3.1 Pre-lymphoma

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).

3.2 Cutaneous T Cell Lymphoma (CTCL)

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).

3.3 Limited Disease (T1/2/3, NP0, M0, B0) 

a) Limited patch or plaque. Those patients whose disease is limited to a single geographical area which can be reasonably encompassed with a single radiation field, may be treated with involved field irradiation.

b)Other limited disease. These patients with either T1, T2 or limited T3 disease are initially treated with either topical Nitrogen Mustard or PUVA. Local radiotherapy to individual symptomatic lesions may be used.

c) Recurrent or progressive or widespread disease (confined to the skin). Those patients who are in adequate health (performance status ECOG 0/1) and who meet the above criteria are recommended to receive radiotherapy to the entire skin (total body electron beam irradiation (TBE)), delivered as a fractionated course over four weeks. Tumor stage lesions are given additional irradiation. This is followed (once the skin reaction has completely subsided) by topical Nitrogen Mustard applied to the entire skin surface daily for one year (see Patient information sheet for the use of topical Nitrogen Mustard (Mechlorethamine)).

3.4 Advanced Disease

Patients presenting with advanced cutaneous disease or systemic involvement (advanced T3 or T4, or NP1+ or NV1+ or M1 or B1) may be treated in a variety of ways. The usual order of sequencing these treatments is as follows:
 

1.	Advanced cutaneous disease
	a) PUVA or topical Nitrogen Mustard (plus radiotherapy to individual symptomatic lesions)
	b) Acitretin
	c) Chlorambucil +/- prednisone
	d) Gemcitabine
	e) Other systemic therapy
2.	Systemic disease
	a) Chlorambucil ± prednisone (plus radiotherapy to symptomatic lesions)
	b) Acitretin (if the pace of progression is slow)
	c) Gemcitabine
	d) Other systemic therapy

Symptomatic or ulcerating lesions usually respond well to local irradiation.
Leukapheresis may be useful where the Sézary cell count exceeds 25,000/ml.
Photopheresis may be of benefit in selected cases.

3.5 Relapsed Disease after TBE, Clinically Limited to the Skin

1. Those patients relapsing after electron beam irradiation often present difficult problems from the point of view of therapy. As with those patients presenting with advanced disease a number of treatments are available (see 3.4.1. above).