Lack of Durable Remission with Conventional-Dose Total Skin Electron Therapy for the Management of Sezary Syndrome and Multiply Relapsed Mycosis Fungoides
Abstract
:1. Introduction
2. Results
2.1. Characterisitics of Eligible Patients
2.2. Conventional-Dose Total Skin Electron Therapy
2.3. Overall Survival of the Whole Cohort
2.4. Overall Survival and Time to Next Treatment in Patients with Sezary Syndrome
2.5. Overall Survival and Time to Next Treatment in Heavily Pre-Treated Patients with Mycosis Fungoides, According to Number of Prior Lines of Therapy
3. Discussion
4. Materials and Methods
5. Conclusions
Author Contributions
Funding
Acknowledgments
Conflicts of Interest
References
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Variable | Total (n = 70) | MF (n = 53) | SS (n = 17) |
---|---|---|---|
Gender | |||
Female | 28 (40.0%) | 19 (35.8%) | 9 (52.9%) |
Male | 42 (60.0%) | 34 (64.2%) | 8 (47.1%) |
Age at diagnosis (years) | |||
Median (range) | 66 (14–81) | 63 (14–81) | 68 (49–81) |
Diagnosis | |||
Mycosis fungoides (MF) | 52 (74.3%) | 52 (98.1%) | 0 (0.0%) |
Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) | 1 (1.4%) | 1 (1.9%) | 0 (0.0%) |
Sezary syndrome (SS) | 17 (24.3%) | 0 (0.0%) | 17 (100.0%) |
Subtype | |||
Folliculotropic | 7 (10.0%) | 7 (13.2%) | 0 (0.0%) |
Poikilodermatous | 1 (1.4%) | 1 (1.9%) | 0 (0.0%) |
Subtype not specified | 62 (88.6%) | 45 (84.9%) | 17 (100.0%) |
Large cell transformation | |||
Prior to cdTSE | 7 (10.0%) | 6 (11.3%) | 1 (5.9%) |
After cdTSE | 11 (15.7%) | 9 (17.0%) | 2 (11.8%) |
Stage at diagnosis (missing n = 1) 1 | |||
IA | 5 (7.1%) | 5 (9.4%) | 0 (0.0%) |
IB | 24 (34.3%) | 23 (43.4%) | 1 (5.9%) |
IIA | 2 (2.9%) | 2 (3.8%) | 0 (0.0%) |
IIB | 17 (24.3%) | 16 (30.2%) | 1 (5.9%) |
III | 10 (14.3%) | 4 (7.5%) | 6 (35.3%) |
IVA1 | 8 (11.4%) | 1 (1.9%) | 7 (41.2%) |
IVA2 | 2 (2.9%) | 1 (1.9%) | 1 (5.9%) |
IVB | 2 (2.9%) | 1 (1.9%) | 1 (5.9%) |
ECOG at diagnosis (missing n = 3) | |||
0 | 57 (85.1%) | 47 (92.2%) | 10 (62.5%) |
1 | 10 (14.9%) | 4 (7.8%) | 6 (37.5%) |
T Stage at cdTSE | |||
1 | 1 (1.4%) | 1 (1.9%) | 0 (0.0%) |
2 | 34 (48.6%) | 32 (60.4%) | 2 (11.8%) 2 |
3 | 15 (21.4%) | 14 (26.4%) | 1 (5.9%) 2 |
4 | 20 (28.6%) | 6 (11.3%) | 14 (82.4%) |
ECOG at cdTSE (missing n = 7) | |||
0 | 36 (57.1%) | 33 (71.7%) | 3 (17.6%) |
1 | 21 (33.3%) | 10 (21.7%) | 11 (64.7%) |
2 | 4 (6.3%) | 2 (4.3%) | 2 (11.8%) |
3 | 2 (3.2%) | 1 (2.2%) | 1 (5.9%) |
Treatment lines prior to cdTSE | |||
Median (range) | 4 (0–14) | 3 (0–14) | 7 (0–13) |
Fractionation schedule | |||
5 fractions per week | 33 (47.1%) | 22 (41.5%) | 11 (64.7%) |
4 fractions per week | 1 (1.4%) | 1 (1.9%) | 0 (0.0%) |
3 fractions per week | 34 (48.6%) | 28 (52.8%) | 6 (35.3%) |
2 fractions per week | 2 (2.9%) | 2 (3.8%) | 0 (0.0%) |
Unscheduled treatment disruptions or failure to complete cdTSE | |||
Yes 3 | 8 (11.4%) | 5 (9.4%) | 3 (17.6%) |
Skin response following cdTSE (missing n = 3) | |||
Overall skin response (complete or partial) | 62 (92.5%) | 48 (92.2%) | 14 (93.8%) |
Stable disease | 4 (6.0%) | 3 (5.9%) | 1 (6.3%) |
Progression | 1 (1.5%) | 1 (2.0%) | 0 (0.0%) |
Therapy | Total (n = 70) | MF (n = 53) | SS (n = 17) |
---|---|---|---|
Phototherapy 1 | 43 (61.4%) | 34 (64.2%) | 9 (52.9%) |
Methotrexate | 34 (48.6%) | 21 (39.6%) | 13 (76.5%) |
Systemic corticosteroids | 32 (45.7%) | 20 (37.7%) | 12 (70.6%) |
Interferon | 23 (32.9%) | 14 (26.4%) | 9 (52.9%) |
Localised radiotherapy | 20 (28.6%) | 19 (35.8%) | 1 (5.9%) |
Multi-agent chemotherapy | 16 (22.9%) | 8 (15.1%) | 8 (47.1%) |
Histone deacetylase (HDAC) inhibitor | 13 (18.6%) | 7 (13.2%) | 6 (35.3%) |
Single-agent chemotherapy | 11 (15.7%) | 7 (13.2%) | 4 (23.5%) |
Extracorporeal photopheresis | 9 (12.9%) | 0 (0%) | 9 (52.9%) |
Haematopoietic stem cell transplant 2 | 5 (7.1%)4 | 3 (5.7%) | 2 (11.8%) |
Immunosuppressant 3 | 5 (7.1%) | 4 (7.5%) | 1 (5.9%) |
Monoclonal antibodies 4 | 5 (7.1%) | 3 (5.7%) | 2 (11.8%) |
Retinoid | 5 (7.1%) | 3 (5.7%) | 2 (11.8%) |
Denileukin difitox | 2 (2.9%) | 0 (0%) | 2 (11.8%) |
Bromodomain and extra-terminal motif (BET) inhibitor | 1 (1.4%) | 1 (1.9%) | 0 (0%) |
Bortezomib | 1 (1.4%) | 1 (1.9%) | 0 (0%) |
No prior therapy (cdTSE first-line) | 7 (10.0%) | 6 (11.3%) | 1 (5.9%) |
Treatment | n | Median TTNT (Months) | TTNT 95% CI (Months) | 1-Year Free from Further Treatment (%) | 2-Years Free from Further Treatment (%) | Median Number of Previous Lines of Therapy |
---|---|---|---|---|---|---|
a-interferon | 68 | 8.7 | 6.0–18.0 | 41.7 | 29.1 | 1 |
Low-dose methotrexate | 83 | 5.0 | 3.6–6.5 | 25.1 | 21.2 | 1 |
Histone deacetylase (HDAC) inhibitors | 74 | 4.5 | 4.0–6.1 | 20.0 | 14.5 | 2 |
Bexarotene | 20 | 7.3 | 2.6–110.8 | 47.4 | 36.8 | 1 |
Alemtuzumab | 16 | 4.1 | 2.7–6.5 | 27.8 | 27.8 | 2.5 |
Denileukin diftitox | 22 | 5.1 | 2.7–6.5 | 22.7 | 22.7 | 3 |
Chemotherapy | 143 | 3.9 | 3.2–5.1 | 10.7 | 5.4 | 3 |
Extracorporeal photophoresis (ECP) | 53 | 9.2 | 5.9–12.8 | 39.1 | 25.7 | 1 |
Autologous SCT | 19 | 7.8 | 4.7–24.4 | 41.5 | 28.4 | 2 |
Allogeneic SCT | 9 | 34.6 | 11.5–NA | 80.0 | 53.3 | 5 |
cdTSE for MF 1 (current study) | 23 | 23.2 | 12.7–34.8 | 76 | 43 | 0–2 |
30 | 7.1 | 3.4–10.9 | 31 | 19 | 3–14 | |
cdTSE for SS (current study) | 17 | 3.7 | 2.3–4.4 | 0 | 0 | 7 |
Skin Stage | Description |
---|---|
T1 | Limited patches, papules, and/or plaques covering <10% of the skin surface. May further stratify into T1a (patch only) vs. T1b (plaque ± patch). |
T2 | Patches, papules or plaques covering ≥10% of the skin surface. May further stratify into T2a (patch only) vs. T2b (plaque ± patch). |
T3 | One or more tumours (≥1 cm diameter) |
T4 | Confluence of erythema covering ≥80% body surface area |
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Campbell, B.A.; Ryan, G.; McCormack, C.; Tangas, E.; Bressel, M.; Twigger, R.; Buelens, O.; van der Weyden, C.; Prince, H.M. Lack of Durable Remission with Conventional-Dose Total Skin Electron Therapy for the Management of Sezary Syndrome and Multiply Relapsed Mycosis Fungoides. Cancers 2019, 11, 1758. https://doi.org/10.3390/cancers11111758
Campbell BA, Ryan G, McCormack C, Tangas E, Bressel M, Twigger R, Buelens O, van der Weyden C, Prince HM. Lack of Durable Remission with Conventional-Dose Total Skin Electron Therapy for the Management of Sezary Syndrome and Multiply Relapsed Mycosis Fungoides. Cancers. 2019; 11(11):1758. https://doi.org/10.3390/cancers11111758
Chicago/Turabian StyleCampbell, Belinda A., Gail Ryan, Christopher McCormack, Eleanor Tangas, Mathias Bressel, Robert Twigger, Odette Buelens, Carrie van der Weyden, and H. Miles Prince. 2019. "Lack of Durable Remission with Conventional-Dose Total Skin Electron Therapy for the Management of Sezary Syndrome and Multiply Relapsed Mycosis Fungoides" Cancers 11, no. 11: 1758. https://doi.org/10.3390/cancers11111758