Isotretinoin |
MF/ SS |
43-80 |
8-33 |
RD: 3-15 months |
2222 Amitay-Laish I, Reiter O, Prag-Naveh H, Kershenovich R, Hodak E. Retinoic acid receptor agonist as monotherapy for early-stage mycosis fungoides: does it work?. J Dermatolog Treat. 2019;30:258-63.,2323 Huen AO, Kim EJ. The Role of Systemic Retinoids in the Treatment of Cutaneous T-Cell Lymphoma. Dermatol Clin. 2015;33:715-29.
|
Acitretin |
Ia-IV |
59-64 |
4-34.6 |
RD: 28 months |
2222 Amitay-Laish I, Reiter O, Prag-Naveh H, Kershenovich R, Hodak E. Retinoic acid receptor agonist as monotherapy for early-stage mycosis fungoides: does it work?. J Dermatolog Treat. 2019;30:258-63.
23 Huen AO, Kim EJ. The Role of Systemic Retinoids in the Treatment of Cutaneous T-Cell Lymphoma. Dermatol Clin. 2015;33:715-29.-2424 Nikolaou V, Patsatsi A, Sidiropoulou P, Chlouverakis G, Kavvalou E, Koletsa T, et al. Monotherapy and combination therapy with acitretin for mycosis fungoides: results of a retrospective, multicenter study. J Eur Acad Dermatol Venereol. 2020;34:2534-40.
|
Bexarotene |
I-IIa |
54-67 |
7-27 |
RD: 9.9-17.2 months |
2323 Huen AO, Kim EJ. The Role of Systemic Retinoids in the Treatment of Cutaneous T-Cell Lymphoma. Dermatol Clin. 2015;33:715-29.,2525 Duvic M, Hymes K, Heald P, Breneman D, Martin A, Myskowski P, et al. Bexarotene is effective and safe for treatment of refractory advanced-stage cutaneous T-cell lymphoma: multinational phase II-III trial results. J Clin Oncol. 2001;19:2456-71.
|
≥IIb |
45-54 |
2-13 |
2323 Huen AO, Kim EJ. The Role of Systemic Retinoids in the Treatment of Cutaneous T-Cell Lymphoma. Dermatol Clin. 2015;33:715-29.,2525 Duvic M, Hymes K, Heald P, Breneman D, Martin A, Myskowski P, et al. Bexarotene is effective and safe for treatment of refractory advanced-stage cutaneous T-cell lymphoma: multinational phase II-III trial results. J Clin Oncol. 2001;19:2456-71.
|
Alpha interferon |
Ia-IVa |
29-80 |
4-67 |
RD: 5-8 months |
2626 Jumbou O, N’Guyen JM, Tessier MH, Legoux B, Dreno B. Long-term follow-up in 51 patients with mycosis fungoides and Sezary syndrome treated by interferon-alfa. Br J Dermatol. 1999;140:427-31.,2929 Spaccarelli N, Rook AH. The Use of Interferons in the Treatment of Cutaneous T-Cell Lymphoma. Dermatol Clin. 2015;33:731-45.
|
Pegylated alpha-interferon |
Ib-III |
50-83 |
33-67 |
- |
2828 Schiller M, Tsianakas A, Sterry W, Dummer R, Hinke A, Nashan D, et al. Dose-escalation study evaluating pegylated interferon alpha-2a in patients with cutaneous T-cell lymphoma. J Eur Acad Dermatol Venereol. 2017;31:1841-7.
|
Pegylated liposomal doxorubicin |
Ib-IV |
41-88 |
6 |
RD: 6-13.3 months |
3232 Wollina U, Dummer R, Brockmeyer NH, Konrad H, Busch JO, Kaatz M, et al. Multicenter study of pegylated liposomal doxorubicin in patients with cutaneous T-cell lymphoma. Cancer. 2003;98:993-1001.,3333 Dummer R, Quaglino P, Becker JC, Hasan B, Karrasch M, Whittaker S, et al. Prospective international multicenter phase II trial of intravenous pegylated liposomal doxorubicin monochemotherapy in patients with stage IIB, IVA, or IVB advanced mycosis fungoides: final results from EORTC 21012. J Clin Oncol. 2012;30:4091-7.
|
Gemcitabine |
T3-T4 |
47-70 |
11.5-22 |
RD: 10-15 months |
3434 Zinzani PL, Venturini F, Stefoni V, Fina M, Pellegrini C, Derenzini E, et al. Gemcitabine as single agent in pretreated T-cell lymphoma patients: evaluation of the long-term outcome. Ann Oncol. 2010;21:860-3.,3838 Hughes C, Khot A, McCormack C, Lade S, Westerman D, Twigger R, et al. Lack of durable disease control with chemotherapy for mycosis fungoides and Sézary syndrome: a comparative study of systemic therapy. Blood. 2015;125:71.
|
Chlorambucil |
SS |
85 |
35 |
RD: 16.5 months |
3636 Winkelmann RK, Diaz-Perez JL, Buechner SA. The treatment of Sezary syndrome. J Am Acad Dermatol. 1984;10:1000-4.
|
III-IVb |
100 |
53.8 |
3535 Coors EA, von den Driesch P. Treatment of erythrodermic cutaneous T-cell lymphoma with intermittent chlorambucil and fluocortolone therapy. Br J Dermatol. 2000;143:127-31.
|
Methotrexate, low dose |
T2 |
33 |
33 |
RD: 15 months |
3939 Zackheim HS, Kashani-Sabet M, McMillan A. Low-dose methotrexate to treat mycosis fungoides: a retrospective study in 69 patients. J Am Acad Dermatol. 2003;49:873-8.
|
III |
56 |
41 |
RD: 31 months |
3939 Zackheim HS, Kashani-Sabet M, McMillan A. Low-dose methotrexate to treat mycosis fungoides: a retrospective study in 69 patients. J Am Acad Dermatol. 2003;49:873-8.
|
Pralatrexate |
IIa-IV |
40.8 |
6.1 |
RD: 6 months |
4040 Wood GS, Wu J. Methotrexate and Pralatrexate. Dermatol Clin. 2015;33:747-55.
|
Romidepsin |
IIb |
34-38 |
6-7 |
RD: 15 months |
4141 Whittaker SJ, Demierre MF, Kim EJ, Rook AH, Lerner A, Duvic M, et al. Final results from a multicenter, international, pivotal study of romidepsin in refractory cutaneous T-cell lymphoma. J Clin Oncol. 2010;28:4485-91.
|
Vorinostat |
Ib-IVa |
30 |
- |
RD: 5 months |
4242 Olsen EA, Kim YH, Kuzel TM, Pacheco TR, Foss FM, Parker S, et al. Phase IIb multicenter trial of vorinostat in patients with persistent, progressive, or treatment refractory cutaneous T-cell lymphoma. J Clin Oncol. 2007;25:3109-15.
|
Alemtuzumab |
adv. MF-SS |
38-84 |
0-47 |
RD: 4 months |
4444 de Masson A, Guitera P, Brice P, Moulonguet I, Mouly F, Bouaziz JD, et al. Long-term efficacy and safety of alemtuzumab in advanced primary cutaneous T-cell lymphomas. Br J Dermatol. 2014;170:720-4.,4545 Bernengo MG, Quaglino P, Comessatti A, Ortoncelli M, Novelli M, Lisa F, et al. Low-dose intermittent alemtuzumab in the treatment of Sezary syndrome: clinical and immunologic findings in 14 patients. Haematologica. 2007;92:784-94.
|
Brentuximab vedotin |
Ia-IVb |
50 |
10 |
TNT: 13.4 months |
4646 Prince HM, Kim YH, Horwitz SM, Dummer R, Scarisbrick J, Quaglino P, et al. Brentuximab vedotin or physician’s choice in CD30-positive cutaneous T-cell lymphoma (ALCANZA): an international, open-label, randomised, phase 3, multicentre trial. Lancet. 2017;390:555-66.
|
Mogamulizumab |
Ia-IVb/SS |
28 (MF: 21; SS: 37) |
- |
PFS: 7.7 months |
4747 Kim YH, Bagot M, Pinter-Brown L, Rook AH, Porcu P, Horwitz SM, et al. Mogamulizumab versus vorinostat in previously treated cutaneous T-cell lymphoma (MAVORIC): an international, open-label, randomised, controlled phase 3 trial. Lancet Oncol. 2018;19:1192-204.
|
CHOP |
IIb |
66 |
- |
TNT: 5.7 months |
3838 Hughes C, Khot A, McCormack C, Lade S, Westerman D, Twigger R, et al. Lack of durable disease control with chemotherapy for mycosis fungoides and Sézary syndrome: a comparative study of systemic therapy. Blood. 2015;125:71.
|
EPOCH |
IIb-IV |
80 |
- |
PFS: 8 months |
3838 Hughes C, Khot A, McCormack C, Lade S, Westerman D, Twigger R, et al. Lack of durable disease control with chemotherapy for mycosis fungoides and Sézary syndrome: a comparative study of systemic therapy. Blood. 2015;125:71.
|
Fludarabine + CFM |
IIb-III |
55 |
- |
RD: 10 months |
3838 Hughes C, Khot A, McCormack C, Lade S, Westerman D, Twigger R, et al. Lack of durable disease control with chemotherapy for mycosis fungoides and Sézary syndrome: a comparative study of systemic therapy. Blood. 2015;125:71.
|
Fludarabine + IFN |
IIa-IVb |
40-58 |
- |
PFS: 5.9 months |
3838 Hughes C, Khot A, McCormack C, Lade S, Westerman D, Twigger R, et al. Lack of durable disease control with chemotherapy for mycosis fungoides and Sézary syndrome: a comparative study of systemic therapy. Blood. 2015;125:71.
|
IFN + PUVA |
Ia-IVa |
80.6 |
75 |
RD: 32 months |
3030 Chiarion-Sileni V, Bononi A, Fornasa CV, Soraru M, Alaibac M, Ferrazzi E, et al. Phase II trial of interferon-alpha-2a plus psolaren with ultraviolet light A in patients with cutaneous T-cell lymphoma. Cancer. 2002;95:569-75.
|
|
|
- |
- |
PFS, 5 years: 75% |
3030 Chiarion-Sileni V, Bononi A, Fornasa CV, Soraru M, Alaibac M, Ferrazzi E, et al. Phase II trial of interferon-alpha-2a plus psolaren with ultraviolet light A in patients with cutaneous T-cell lymphoma. Cancer. 2002;95:569-75.
|
|
Ia-IIa |
98 |
84 |
PFS, 5 years: 27% |
3131 Rupoli S, Goteri G, Pulini S, Filosa A, Tassetti A, Offidani M, et al. Long-term experience with low-dose interferon-alpha and PUVA in the management of early mycosis fungoides. Eur J Haematol. 2005;75:136-45.
|
IFN peg + PUVA |
Ia-IV |
88 |
44 |
PFS: 30.9 months |
2727 Husken AC, Tsianakas A, Hensen P, Nashan D, Loquai C, Beissert S, et al. Comparison of pegylated interferon alpha-2b plus psoralen PUVA versus standard interferon alpha-2a plus PUVA in patients with cutaneous T-cell lymphoma. J Eur Acad Dermatol Venereol. 2012;26:71-8.
|
IFN + retinoid |
Ia-IIb |
38 |
6 |
- |
2929 Spaccarelli N, Rook AH. The Use of Interferons in the Treatment of Cutaneous T-Cell Lymphoma. Dermatol Clin. 2015;33:731-45.
|
Bexaroteno + PUVA |
Ib-IIa |
77 |
31 |
RD: 5.8 months |
5252 Whittaker S, Ortiz P, Dummer R, Ranki A, Hasan B, Meulemans B, et al. Efficacy and safety of bexarotene combined with psoralen-ultraviolet A (PUVA) compared with PUVA treatment alone in stage IB-IIA mycosis fungoides: final results from the EORTC Cutaneous Lymphoma Task Force phase III randomized clinical trial (NCT00056056). Br J Dermatol. 2012;167:678-87.
|
Isolated ECP |
III-IVa1 |
24 |
9 |
TNT: 14 months |
4949 Olsen EA, Rook AH, Zic J, Kim Y, Porcu P, Querfeld C, et al. Sezary syndrome: immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC). J Am Acad Dermatol. 2011;64:352-404.
50 Zic JA. Extracorporeal Photopheresis in the Treatment of Mycosis Fungoides and Sezary Syndrome. Dermatol Clin. 2015;33:765-76.-5151 Gao C, McCormack C, van der Weyden C, Goh MS, Campbell BA, Twigger R, et al. Prolonged survival with the early use of a novel extracorporeal photopheresis regimen in patients with Sezary syndrome. Blood. 2019;134:1346-50.
|
ECP + IFN |
III-IVa1 |
45.4 |
18.2 |
- |
4949 Olsen EA, Rook AH, Zic J, Kim Y, Porcu P, Querfeld C, et al. Sezary syndrome: immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC). J Am Acad Dermatol. 2011;64:352-404.,5050 Zic JA. Extracorporeal Photopheresis in the Treatment of Mycosis Fungoides and Sezary Syndrome. Dermatol Clin. 2015;33:765-76.
|
ECP + IFN + Bexarotene |
III-IVa1 |
88.2 |
32.4 |
RD: 4 months |
4949 Olsen EA, Rook AH, Zic J, Kim Y, Porcu P, Querfeld C, et al. Sezary syndrome: immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC). J Am Acad Dermatol. 2011;64:352-404.,5050 Zic JA. Extracorporeal Photopheresis in the Treatment of Mycosis Fungoides and Sezary Syndrome. Dermatol Clin. 2015;33:765-76.
|