Relevant outcomes

• Severity of symptoms (pruritus, sleep disturbance, pain) and signs (erythema, scaling, fissuring, excoriation, oedema and thickness of plaques, presence of nodules, tumours, peripheral lymphadenopathy)

• Body surface area involvement

• Assessment of overall tumour burden, with histological assessment of skin and lymph nodes, staging CT scans and peripheral blood Sezary cell counts/lymphocyte subsets

• Establishment of molecular remission using T-cell receptor gene analysis of skin and peripheral blood

IA IB IIA IIB III IVA IVB Overall Reference No. of Median patients follow up

IA IB IIA IIB III IVA IVB Overall Reference No. of Median patients follow up

Overall survival (%)

99

86

49

65

40

0

80

Doom et al. 2000aa

309

5-2

5 years

100

84

52

57

Zackheim eia/, 19993b

489

4-7

96

(78)

(40)

(40)

Kim eia/. 1996'3C

122

9-8

73

73d

Kim eia/. 1999™

176

8

45

17

Kim eia/. 199512e

106

10-5

15

15

Coninck et al. 2001111

112

10 years

84

61

49

27

20

0

57

Doom et al. 20003

309

5-2

100

67

39

41

Zackheim eia/, 19993

489

4-7

88

(60)

(20)

(20)

Kim eia/. 19963

122

9-8

58

45d

Kim eia/. 199910

176

8

5

5

Connlck et al. 200111

112

Disease-free survival (%)

5 years

100

96

68

80

40

0

89

Doom et al. 20003

309

5-2

10 years

97

83

68

42

20

0

75

Doom et al. 20003

309

5-2

98

Kim etal. 19963

122

9-8

Median survival (years)

NRC

12' 1

29

3-6

Kim etal. 1996-

556

9-8

128

10-0

Kim etal. 199910

176

8

4-6

13 mos

Kim etal. 1995'2

106

10-5

3 mos

13 mos

Coninck etal. 200111

546

Disease progression (%)

5 years

4

21

65

32

70

100

Doom et al. 20003

309

5-2

10 years

10

39

65

60

70

100

Doom et al. 20003

309

5-2

20 years

0

10

36

41

Coninck etal. 200111

546

IA

IB

IIA

IIB I

II IVA

IVB

Overall Reference

No. of patients

Median follow up (years)

Overall disease progression

9

20

34d

Kim et al. 19963 Kim et al. 199910

122 176

9-8 8

FFR (%)

5 years

50

36

9

Kim et al. 19963 Kim et al. 199910

122 176

9-8 8

10 years

25 (50)c

31

3

Kim et al. 19963 Kim et al. 199910

122 176

9-8 8

All overall (OS) survival curves were calculated using the Kaplan-Meier method.

aln the study by Doom eta!.3 the presence of follicular mucinosis was an Independent poor prognostic feature possibly related to depth of Infiltrate In patients with stage IB disease (disease-free survival of 81% and 36% and OS of 75% and 21% at 5 and 10 years, respectively). A lack of a complete response to Initial therapy was also associated with a poor outcome (P<0.001) In a multivariate analysis as well as Increasing clinical stage and the presence of extracutaneous disease. A different staging system was used In this study (Hammlnga et al. Br J Dermatol 1982;107:145-55) but for the purposes of this table the staging has been altered to be consistent. This study Is the only one to provide comprehensive disease-specific survival (DSS) data for different stages of mycosis fungoldes. Only three patients had stage IVB disease and only 18 patients each had stage IIA and IVA disease. Therefore the results for these stages must be Interpreted cautiously.

bln the study by Zackhelm et al.3 black patients had a relatively more advanced stage of disease than white patients. The TNM classification was used In this study. Lymph node stage had an unfavourable Impact on survival but this trend did not reach significance for each IndividualT stage because of a lack of sufficient power (an estimated 1700 subjects required) and IIA/IVA patients were not designated separately. Similar considerations apply to peripheral blood Involvement. Similar outcomes for patients with stage IIB (T3) and III (T4) disease Is consistent with other studies but this might reflect a lack of lymph node staging data Included In this study.

The 1996 study by Kim et al.'- primarily Included data on 122 patients with stage IA disease, but survival data on 556 patients with all stages were also Included to give the values In parentheses. The freedom from relapse (FFR) data at 5 and 10 years are confusing because the text states that the FFR at 10 years was 25% but the figure Indicates that It remains at approximately 50%, as for FFR at 5 years. The median survival for stage IA patients was not reached at 32-5 years. NR, not reached dln the 1999 study by Kim et al.m OS at 20 years for stage IB and IIA patients was 27%. DSS was better for patients <58 years of age (P<0-03). In 23 of the 56 patients with palpable lymphadenopathy, no histological assessment was made and these patients were assumed to have reactlve/dermatopathlc nodes (IIA). This might account for the lack of difference In OS at 5 years between stage IB and IIA patients, although there appears to be a difference In OS at 10 years.

eln the 1995 study by Kim ei a/.12 the OS and median survival data was calculated from the date of Initial treatment, which was usually within 3 months of diagnosis. This study also stratified patients Into three groups according to the presence of none, one, or two or three poor prognostic parameters, namely: age at presentation (>65 years), the presence of clinical adenopathy, and B1 stage, producing varied median survivals of 10^2 years (no factors), 37 years (one factor) and 1-5 years (two or three factors) P<0-005. The study by Connlck eta!" Included 112 patients with extracutaneous disease at presentation or with progression and 434 patients with only cutaneous disease, giving the 546 patients listed In the table for median survival and disease progression.

Several recent trials have used various scoring systems involving computed measures of the above but most studies included in this review define responses in terms of simple clinical observation with CR defined as complete resolution of clinically apparent disease (based in CTCL usually on cutaneous signs of disease) for at least 4-6 weeks. Partial response (PR) is usually defined as >50% reduction of clinical disease or tumour burden, although some studies in CTCL have defined this as >25% reduction in tumour burden. More importantly, most studies do not include a validated scoring system, which effectively makes any interpretation of PR impossible. Similar considerations apply to assessment of stable disease (SD), defined usually as <50% improvement, and progressive disease (PD), defined as >25% increase in tumour burden. For most studies in CTCL, PD is defined as a deterioration in clinical stage of disease.

Was this article helpful?

0 0
Natural Treatments For Psoriasis

Natural Treatments For Psoriasis

Do You Suffer From the Itching and Scaling of Psoriasis? Or the Chronic Agony of Psoriatic Arthritis? If so you are not ALONE! A whopping three percent of the world’s populations suffer from either condition! An incredible 56 million working hours are lost every year by psoriasis sufferers according to the National Psoriasis Foundation.

Get My Free Ebook


Post a comment