Blood

B0 — no peripheral blood Sezary cells (<5%) B1 — peripheral blood Sezary cells (>5% of total lymphocyte count)

Clinical staging system for CTCL (mycosis fungoides)

Clinical Stages

T

N

M

IA

T1

N0

M0

IB

T2

N0

M0

IIA

T1-2

N1

M0

IIB

T3

N0-1

M0

III

T4

N0-1

M0

IVA

T1-4

N2-3

M0

IVB

T1-4

N0-3

M1

Stage IB patients have an OS rate of 73-86% at 5 years and 58-67% at 10 years, and DSS rates of 96% and 83% at 5 and 10 years respectively.3,8,10 A median survival of 12-1-12-8 years should be expected for stage IB patients, with a risk of disease progression varying from 10% to 39%. The explanation for this marked variation in different studies of stage IB is unclear but it appears that patients with folliculotropic variants of mycosis fungoides have a worse prognosis than other patients with stage IB disease; this may reflect the depth of infiltrate, which perhaps makes skin-directed therapy less effective.8

Accurate data on stage IIA patients (patches/plaques and clinical adenopathy with no histological evidence of lymphoma) are scant, but this stage may be associated with a worse outcome - with OS rates of 49%, DSS rates of 68% and risks of disease progression of 65% at both 5 and 10 years. The lack of difference in outcome at 5 and 10 years in this study is unreliable since the data came from only 18 patients.8 Of the 176 patients reported by Kim etal.10 56 (32%) had peripheral adenopathy (stage IIA) but in 23 of these 56 no histological assessment was made and therefore some of them could have had stage IVA disease. Nevertheless the OS rate is similar to stage IB disease at 5 years (73%), with a slight difference at 10 years (45% versus 58%), associated with a small difference in median survival of 10 years (stage IIA) compared with 12-8 years (stage IB) and overall risk of disease progression of 34% and 20%, respectively. Further studies of larger numbers of patients with stage IIA disease are needed to compare outcomes with those of stage IB patients.

Patients with tumour-stage disease (stage IIB) have OS rates of 40-65% at 5 years and 20-39% at 10 years,3,8,9 and a median survival of 2-9 years in one study.9 In one study DSS rates of 80% and

42% at 5 and 10 years, respectively, were reported for patients with stage IIB disease.8 The survival data for patients with erythrodermic mycosis fungoides but no evidence of lymph node or peripheral blood involvement (stage III) are broadly similar to those for stage IIB disease, although median survival may be better (4-6 versus 3-6 years).3,9,12 In contrast the OS and DSS rates at 5 and 10 years for stage IVA and IVB patients is poor (15-40% and 5-20% for stage IVA and 0-15% and 0-5% for stage IVB at 5 and 10 years, respectively) and a median survival of 13 months for both extracutaneous stages.8,11,12

The OS rates in CTCL based on stage of disease has led to suggestions that the staging system should be modified with four broad categories.13 (i) Stage IA patients have a normal life expectancy but (ii) stage IB and IIA disease may have a similar prognosis, although the thickness of the infiltrate is an important prognostic factor in this group. Similarly (iii) the prognosis is similar for stage IIB and III disease, which is better than in patients with (iv) nodal or visceral disease (IVA/B). This proposal resembles that suggested by Sausville et al. in 1988.14

Patients with Sezary syndrome have an 11% 5-year survival, with a median survival of 32 months from diagnosis.1 In contrast, other clinicopathological variants of CTCL are generally associated with an excellent long-term prognosis (100% 5-year survival in lymphomatoid papulosis and 90% in primary cutaneous CD30+ large cell anaplastic cutaneous lymphoma), with the exception of patients with subcutaneous panniculitis-like T-cell lymphomas and primary cutaneous natural-killer (NK)-like T-cell/NK cell lymphomas.1

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