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Section editor
	Bhagirathbhai Dholaria, MBBS Vanderbilt University Nashville, TN

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16 regimens on this page 19 variants on this page

Guidelines

ESMO

2018: Willemze et al. Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

"How I Treat"

2019: Shinohara & Shustov How I treat primary cutaneous CD30 + lymphoproliferative disorders

NCCN

Upfront therapy

PUVA

PUVA: Psoralen & Ultra-Violet A

Regimen

Study	Years of enrollment	Evidence	Comparator	Comparative Efficacy
Edelson et al. 1987	NR	Non-randomized (RT)
Whittaker et al. 2012 (EORTC 21011)	2003-2010	Phase 3 (C)	PUVA & Bexarotene	Did not meet primary endpoint of ORR

Note: to our knowledge, this regimen was not tested as an experimental arm in an RCT in this context, prior to becoming a standard comparator arm.

Chemotherapy

Methoxsalen (Uvadex) (see papers for details)

References

Edelson R, Berger C, Gasparro F, Jegasothy B, Heald P, Wintroub B, Vonderheid E, Knobler R, Wolff K, Plewig G, McKiernan G, Christiansen I, Oster M, Honigsmann H, Wilford H, Kokoschka E, Rehle T, Perez M, Stingl G, Laroche L. Treatment of cutaneous T-cell lymphoma by extracorporeal photochemotherapy: preliminary results. N Engl J Med. 1987 Feb 5;316(6):297-303. link to original article PubMed
EORTC 21011: Whittaker S, Ortiz P, Dummer R, Ranki A, Hasan B, Meulemans B, Gellrich S, Knobler R, Stadler R, Karrasch M. 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 Sep;167(3):678-87. link to original article PubMed NCT00056056

Topical therapy

Regimen

Study	Years of enrollment	Evidence	Comparator	Comparative Efficacy
Kaye et al. 1989	1979-1987	Randomized, >20 pts (C)	CAV-E & RT	Did not meet primary endpoint of OS60

Usually consists of high-dose topical steroids or nitrogen mustards; see paper for details.

References

Kaye FJ, Bunn PA Jr, Steinberg SM, Stocker JL, Ihde DC, Fischmann AB, Glatstein EJ, Schechter GP, Phelps RM, Foss FM, Parlette HL, Anderson MJ, Sausville EA. A randomized trial comparing combination electron-beam radiation and chemotherapy with topical therapy in the initial treatment of mycosis fungoides. N Engl J Med. 1989 Dec 28;321(26):1784-90. link to original article PubMed

Relapsed or refractory

Alemtuzumab monotherapy

Regimen

Study	Evidence	Efficacy
Lundin et al. 2003	Phase 2	ORR: 55%

Targeted therapy

Alemtuzumab (Campath) 3 mg IV once on day 1, then increased to 10 mg IV once as soon as infusion-related reactions tolerated, then increased to 30 mg IV once as soon as infusion-related reactions tolerated, then 30 mg IV 3 days per week

Up to 12-week course

References

Lundin J, Hagberg H, Repp R, Cavallin-Ståhl E, Fredén S, Juliusson G, Rosenblad E, Tjønnfjord G, Wiklund T, Osterborg A. Phase 2 study of alemtuzumab (anti-CD52 monoclonal antibody) in patients with advanced mycosis fungoides/Sezary syndrome. Blood. 2003 Jun 1;101(11):4267-72. Epub 2003 Jan 23. link to original article contains dosing details in abstract PubMed

Belinostat monotherapy

Regimen

Study	Evidence	Efficacy
Foss et al. 2014 (PXD101-CLN-6)	Phase 2	ORR: 14%

Targeted therapy

Belinostat (Beleodaq) 1000 mg/m² IV over 30 minutes once per day on days 1 to 5

21-day cycles

References

PXD101-CLN-6: Foss F, Advani R, Duvic M, Hymes KB, Intragumtornchai T, Lekhakula A, Shpilberg O, Lerner A, Belt RJ, Jacobsen ED, Laurent G, Ben-Yehuda D, Beylot-Barry M, Hillen U, Knoblauch P, Bhat G, Chawla S, Allen LF, Pohlman B. A phase II trial of belinostat (PXD101) in patients with relapsed or refractory peripheral or cutaneous T-cell lymphoma. Br J Haematol. 2015 Mar;168(6):811-9. Epub 2014 Nov 17. link to original article contains dosing details in abstract PubMed NCT00274651

Bendamustine monotherapy

Regimen

Study	Evidence	Efficacy
Demaj et al. 2013 (BENTLY)	Phase 2	ORR: 50%

Chemotherapy

Bendamustine 120 mg/m² IV once per day on days 1 & 2

21-day cycle for 6 cycles

References

BENTLY: Damaj G, Gressin R, Bouabdallah K, Cartron G, Choufi B, Gyan E, Banos A, Jaccard A, Park S, Tournilhac O, Schiano-de Collela JM, Voillat L, Joly B, Le Gouill S, Saad A, Cony-Makhoul P, Vilque JP, Sanhes L, Schmidt-Tanguy A, Bubenheim M, Houot R, Diouf M, Marolleau JP, Béné MC, Martin A, Lamy T. Results from a prospective, open-label, phase II trial of bendamustine in refractory or relapsed T-cell lymphomas: the BENTLY trial. J Clin Oncol. 2013 Jan 1;31(1):104-10. Epub 2012 Oct 29. link to original article contains dosing details in manuscript PubMed NCT00959686

Bexarotene monotherapy

Regimen variant #1, 48 weeks

Study	Years of enrollment	Evidence	Comparator	Comparative Efficacy
Prince et al. 2017 (ALCANZA)	2012-2015	Phase 3 (C)	Brentuximab vedotin	Inferior PFS¹

¹Reported efficacy is based on the 2021 update.
Note: This dose is considered the target dose in ALCANZA.

Targeted therapy

Bexarotene (Targretin) 300 mg/m² PO once per day

48-week course

Regimen variant #2, indefinite

Study	Years of enrollment	Evidence	Comparator	Comparative Efficacy
Duvic et al. 2001a	1996-1998	Phase 2 (RT)		ORR: 55%
Duvic et al. 2001b	1997-1998	Phase 2/3 (E-RT-esc)	Bexarotene; 6.5 mg/m²/d	Superior ORR

Note: This dose is considered the optimal starting dose by Duvic et al. 2001a.

Targeted therapy

Bexarotene (Targretin) 300 mg/m² PO once per day

Continued indefinitely

References

Duvic M, Hymes K, Heald P, Breneman D, Martin AG, Myskowski P, Crowley C, Yocum RC; Bexarotene Worldwide Study Group. 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 May 1;19(9):2456-71. link to original article contains dosing details in manuscript PubMed
Duvic M, Martin AG, Kim Y, Olsen E, Wood GS, Crowley CA, Yocum RC; Worldwide Bexarotene Study Group. Phase 2 and 3 clinical trial of oral bexarotene (Targretin capsules) for the treatment of refractory or persistent early-stage cutaneous T-cell lymphoma. Arch Dermatol. 2001 May;137(5):581-93. link to original article contains dosing details in manuscript PubMed
ALCANZA: Prince HM, Kim YH, Horwitz SM, Dummer R, Scarisbrick J, Quaglino P, Zinzani PL, Wolter P, Sanches JA, Ortiz-Romero PL, Akilov OE, Geskin L, Trotman J, Taylor K, Dalle S, Weichenthal M, Walewski J, Fisher D, Dréno B, Stadler R, Feldman T, Kuzel TM, Wang Y, Palanca-Wessels MC, Zagadailov E, Trepicchio WL, Zhang W, Lin HM, Liu Y, Huebner D, Little M, Whittaker S, Duvic M; ALCANZA study group. 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 Aug 5;390(10094):555-566. Epub 2017 Jun 7. link to original article contains dosing details in manuscript PubMed NCT01578499
1. Update: Horwitz SM, Scarisbrick JJ, Dummer R, Whittaker S, Duvic M, Kim YH, Quaglino P, Zinzani PL, Bechter O, Eradat H, Pinter-Brown L, Akilov OE, Geskin L, Sanches JA, Ortiz-Romero PL, Weichenthal M, Fisher DC, Walewski J, Trotman J, Taylor K, Dalle S, Stadler R, Lisano J, Bunn V, Little M, Prince HM. Randomized phase 3 ALCANZA study of brentuximab vedotin vs physician's choice in cutaneous T-cell lymphoma: final data. Blood Adv. 2021 Dec 14;5(23):5098-5106. link to original article link to PMC article PubMed

Bexarotene & Pralatrexate

Regimen

Study	Years of enrollment	Evidence	Efficacy
Duvic et al. 2017 (PDX-018)	2010-2015	Phase 1/2	ORR: 60%

Note: This dose is the MTD. Note that the abstract contains a typo for bexarotene dosing; the authors have been contacted.

Targeted therapy

Bexarotene (Targretin) 150 mg/m² PO once per day

Chemotherapy

Pralatrexate (Folotyn) 15 mg/m² IV once per day on days 1, 8, 15

28-day cycles

References

PDX-018: Duvic M, Kim YH, Zinzani PL, Horwitz SM. Results from a phase I/II open-label, dose-finding study of pralatrexate and oral bexarotene in patients with relapsed/refractory cutaneous T-cell lymphoma. Clin Cancer Res. 2017 Jul;23(14):3552-6. Epub 2017 Feb 6. link to original article contains dosing details in manuscript link to PMC article PubMed NCT01134341

Brentuximab vedotin monotherapy

Regimen

Study	Years of enrollment	Evidence	Comparator	Comparative Efficacy
Kim et al. 2015 (SU-06212011-7946)	NR	Phase 2		OGRR: 70% (90% CI, 53-83)
Duvic et al. 2015 (MDACC 2010-0914)	2011-2013	Phase 2		ORR: 73% (95% CI, 60-86)
Prince et al. 2017 (ALCANZA)	2012-2015	Phase 3 (E-RT-switch-ooc)	Investigator's choice of: 1. Bexarotene 2. Methotrexate	Superior PFS¹ Median PFS: 16.7 vs 3.5 mo (HR 0.27, 95% CI 0.17-0.43)

¹Reported efficacy is based on the 2021 update.

Antibody-drug conjugate therapy

Brentuximab vedotin (Adcetris) 1.8 mg/kg IV over 30 minutes once on day 1

21-day cycle for up to 16 cycles

References

SU-06212011-7946: Kim YH, Tavallaee M, Sundram U, Salva KA, Wood GS, Li S, Rozati S, Nagpal S, Krathen M, Reddy S, Hoppe RT, Nguyen-Lin A, Weng WK, Armstrong R, Pulitzer M, Advani RH, Horwitz SM. Phase II investigator-initiated study of brentuximab vedotin in mycosis fungoides and Sézary syndrome with variable CD30 expression level: A multi-institution collaborative project. J Clin Oncol. 2015 Nov 10;33(32):3750-8. Epub 2015 Jul 20. link to original article contains dosing details in manuscript link to PMC article PubMed NCT01396070
MDACC 2010-0914: Duvic M, Tetzlaff MT, Gangar P, Clos AL, Sui D, Talpur R. Results of a phase II trial of brentuximab vedotin for CD30+ cutaneous T-cell lymphoma and lymphomatoid papulosis. J Clin Oncol. 2015 Nov 10;33(32):3759-65. Epub 2015 Aug 10. link to original article contains dosing details in manuscript link to PMC article PubMed NCT01352520
ALCANZA: Prince HM, Kim YH, Horwitz SM, Dummer R, Scarisbrick J, Quaglino P, Zinzani PL, Wolter P, Sanches JA, Ortiz-Romero PL, Akilov OE, Geskin L, Trotman J, Taylor K, Dalle S, Weichenthal M, Walewski J, Fisher D, Dréno B, Stadler R, Feldman T, Kuzel TM, Wang Y, Palanca-Wessels MC, Zagadailov E, Trepicchio WL, Zhang W, Lin HM, Liu Y, Huebner D, Little M, Whittaker S, Duvic M; ALCANZA study group. 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 Aug 5;390(10094):555-566. Epub 2017 Jun 7. link to original article contains dosing details in manuscript PubMed NCT01578499
1. Update: Horwitz SM, Scarisbrick JJ, Dummer R, Whittaker S, Duvic M, Kim YH, Quaglino P, Zinzani PL, Bechter O, Eradat H, Pinter-Brown L, Akilov OE, Geskin L, Sanches JA, Ortiz-Romero PL, Weichenthal M, Fisher DC, Walewski J, Trotman J, Taylor K, Dalle S, Stadler R, Lisano J, Bunn V, Little M, Prince HM. Randomized phase 3 ALCANZA study of brentuximab vedotin vs physician's choice in cutaneous T-cell lymphoma: final data. Blood Adv. 2021 Dec 14;5(23):5098-5106. link to original article link to PMC article PubMed

Denileukin diftitox monotherapy

Regimen

Study	Years of enrollment	Evidence	Comparator	Comparative Efficacy
Olsen et al. 2001 (L4389-10)	NR	Phase 3 (E-RT-esc)	Denileukin diftitox; 9 mcg/kg	Did not meet primary endpoint of ORR
Prince et al. 2010 (L4389-11)	NR	Phase 3 (E-RT-esc)	1. Denileukin diftitox; 9 mcg/kg	Superior ORR
Prince et al. 2010 (L4389-11)	NR	Phase 3 (E-RT-esc)	2. Placebo	Superior PFS

Note: Dose is that which was recommended in L4389-11 based on superior response. Up to 3 additional cycles allowed in L4389-10 for patients who had ongoing response.

Targeted therapy

Denileukin diftitox (Ontak) 18 mcg/kg IV over 15 to 60 minutes once per day on days 1 to 5

Supportive therapy

"Premedication with Acetaminophen (Tylenol) (650 mg in Olsen et al. 2001) and an antihistamine was required 30 to 60 minutes before each infusion" and could be used after infusions as needed.
Olsen et al. 2001 used Promethazine (Phenergan) 25 mg or Prochlorperazine (Compazine) 10 mg (route/schedule not specified) as needed for nausea.
Corticosteroid use was not allowed.

21-day cycles for up to 8 cycles (see note)

References

L4389-10: Olsen E, Duvic M, Frankel A, Kim Y, Martin A, Vonderheid E, Jegasothy B, Wood G, Gordon M, Heald P, Oseroff A, Pinter-Brown L, Bowen G, Kuzel T, Fivenson D, Foss F, Glode M, Molina A, Knobler E, Stewart S, Cooper K, Stevens S, Craig F, Reuben J, Bacha P, Nichols J. Pivotal phase III trial of two dose levels of denileukin diftitox for the treatment of cutaneous T-cell lymphoma. J Clin Oncol. 2001 Jan 15;19(2):376-88. link to original article contains dosing details in manuscript PubMed
L4389-11: Prince HM, Duvic M, Martin A, Sterry W, Assaf C, Sun Y, Straus D, Acosta M, Negro-Vilar A. Phase III placebo-controlled trial of denileukin diftitox for patients with cutaneous T-cell lymphoma. J Clin Oncol. 2010 Apr 10;28(11):1870-7. Epub 2010 Mar 8. link to original article contains dosing details in manuscript PubMed NCT00050999
Meta-analysis: Duvic M, Geskin L, Prince HM. Duration of response in cutaneous T-cell lymphoma patients treated with denileukin diftitox: results from 3 phase III studies. Clin Lymphoma Myeloma Leuk. 2013 Aug;13(4):377-84. Epub 2013 Jun 14. link to original article PubMed

Lenalidomide monotherapy

Regimen

Study	Evidence	Efficacy
Querfeld et al. 2013 (NU 04H5)	Phase 2	ORR: 28%

Targeted therapy

Lenalidomide (Revlimid) as follows:
- Cycle 1: 10 mg PO once per day on days 1 to 21
- Cycle 2, if tolerated: 15 mg PO once per day on days 1 to 21
- Cycle 3, if tolerated: 20 mg PO once per day on days 1 to 21
- Cycle 4 onwards, if tolerated: 25 mg PO once per day on days 1 to 21

28-day cycle for up to 26 cycles (2 years)

References

NU 04H5: Querfeld C, Rosen ST, Guitart J, Duvic M, Kim YH, Dusza SW, Kuzel TM. Results of an open-label multicenter phase II trial of lenalidomide monotherapy in refractory mycosis fungoides and Sezary syndrome. Blood. 2014 Feb 20;123(8):1159-66. Epub 2013 Dec 11. link to original article contains dosing details in manuscript PubMed NCT00466921

Methotrexate monotherapy

Regimen

Study	Years of enrollment	Evidence	Comparator	Comparative Efficacy
Prince et al. 2017 (ALCANZA)	2012-2015	Phase 3 (C)	Brentuximab vedotin	Inferior PFS¹

¹Reported efficacy is based on the 2021 update.
Note: to our knowledge, this regimen was not tested as an experimental arm in an RCT in this context, prior to becoming a standard comparator arm.

Chemotherapy

Methotrexate (MTX) 5 to 50 mg PO once per day on days 1, 8, 15, 22

28-day cycle for 12 cycles

References

Retrospective: 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 Nov;49(5):873-8. link to original article PubMed
ALCANZA: Prince HM, Kim YH, Horwitz SM, Dummer R, Scarisbrick J, Quaglino P, Zinzani PL, Wolter P, Sanches JA, Ortiz-Romero PL, Akilov OE, Geskin L, Trotman J, Taylor K, Dalle S, Weichenthal M, Walewski J, Fisher D, Dréno B, Stadler R, Feldman T, Kuzel TM, Wang Y, Palanca-Wessels MC, Zagadailov E, Trepicchio WL, Zhang W, Lin HM, Liu Y, Huebner D, Little M, Whittaker S, Duvic M; ALCANZA study group. 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 Aug 5;390(10094):555-566. Epub 2017 Jun 7. link to original article contains dosing details in manuscript PubMed NCT01578499
1. Update: Horwitz SM, Scarisbrick JJ, Dummer R, Whittaker S, Duvic M, Kim YH, Quaglino P, Zinzani PL, Bechter O, Eradat H, Pinter-Brown L, Akilov OE, Geskin L, Sanches JA, Ortiz-Romero PL, Weichenthal M, Fisher DC, Walewski J, Trotman J, Taylor K, Dalle S, Stadler R, Lisano J, Bunn V, Little M, Prince HM. Randomized phase 3 ALCANZA study of brentuximab vedotin vs physician's choice in cutaneous T-cell lymphoma: final data. Blood Adv. 2021 Dec 14;5(23):5098-5106. link to original article link to PMC article PubMed

Mogamulizumab monotherapy

Regimen variant #1, 8-week course

Study	Evidence
Ogura et al. 2014 (KW-0761-004)	Phase 2

Targeted therapy

Mogamulizumab (Poteligeo) 1 mg/kg IV once per day on days 1, 8, 15, 22

28-day cycle for 2 cycles

Regimen variant #2, indefinite

FDA-recommended dose

Study	Years of enrollment	Evidence	Comparator	Comparative Efficacy
Duvic et al. 2015 (KW-0761-001)	2009-NR	Phase 1/2
Kim et al. 2018 (MAVORIC)	2012-2016	Phase 3 (E-RT-switch-ooc)	Vorinostat	Superior PFS

Note: in KW-0761-001, a two-week "period of observation" was undertaken after cycle 1.

Targeted therapy

Mogamulizumab (Poteligeo) as follows:
- Cycle 1: 1 mg/kg IV once per day on days 1, 8, 15, 22
- Cycle 2 onwards: 1 mg/kg IV once per day on days 1 & 15

28-day cycles

References

KW-0761-004: Ogura M, Ishida T, Hatake K, Taniwaki M, Ando K, Tobinai K, Fujimoto K, Yamamoto K, Miyamoto T, Uike N, Tanimoto M, Tsukasaki K, Ishizawa K, Suzumiya J, Inagaki H, Tamura K, Akinaga S, Tomonaga M, Ueda R. Multicenter phase II study of mogamulizumab (KW-0761), a defucosylated anti-cc chemokine receptor 4 antibody, in patients with relapsed peripheral T-cell lymphoma and cutaneous T-cell lymphoma. J Clin Oncol. 2014 Apr 10;32(11):1157-63. Epub 2014 Mar 10. link to original article contains dosing details in abstract PubMed NCT01192984
KW-0761-001: Duvic M, Pinter-Brown LC, Foss FM, Sokol L, Jorgensen JL, Challagundla P, Dwyer KM, Zhang X, Kurman MR, Ballerini R, Liu L, Kim YH. Phase 1/2 study of mogamulizumab, a defucosylated anti-CCR4 antibody, in previously treated patients with cutaneous T-cell lymphoma. Blood. 2015 Mar 19;125(12):1883-9. Epub 2015 Jan 20. link to original article contains dosing details in abstract PubMed NCT00888927
MAVORIC: Kim YH, Bagot M, Pinter-Brown L, Rook AH, Porcu P, Horwitz SM, Whittaker S, Tokura Y, Vermeer M, Zinzani PL, Sokol L, Morris S, Kim EJ, Ortiz-Romero PL, Eradat H, Scarisbrick J, Tsianakas A, Elmets C, Dalle S, Fisher DC, Halwani A, Poligone B, Greer J, Fierro MT, Khot A, Moskowitz AJ, Musiek A, Shustov A, Pro B, Geskin LJ, Dwyer K, Moriya J, Leoni M, Humphrey JS, Hudgens S, Grebennik DO, Tobinai K, Duvic M; MAVORIC Investigators. Mogamulizumab versus vorinostat in previously treated cutaneous T-cell lymphoma (MAVORIC): an international, open-label, randomised, controlled phase 3 trial. Lancet Oncol. 2018 Sep;19(9):1192-1204. Epub 2018 Aug 9. link to original article contains dosing details in abstract PubMed NCT01728805

Pralatrexate monotherapy

Regimen

Study	Evidence	Efficacy
Horwitz et al. 2012	Phase 2	RR: 45%

Note: Dose is that identified as recommended based on de-escalation strategy.

Chemotherapy

Pralatrexate (Folotyn) 15 mg/m² IV push once per day on days 1, 8, 15

Supportive therapy

Cyanocobalamin (Vitamin B12) 1 mg IM once every 8 to 10 weeks, within 10 weeks of treatment initiation
Folic acid (Folate) 1 mg PO once per day, starting at least 10 days prior to treatment initiation

28-day cycles

References

Horwitz SM, Kim YH, Foss F, Zain JM, Myskowski PL, Lechowicz MJ, Fisher DC, Shustov AR, Bartlett NL, Delioukina ML, Koutsoukos T, Saunders ME, O'Connor OA, Duvic M. Identification of an active, well-tolerated dose of pralatrexate in patients with relapsed or refractory cutaneous T-cell lymphoma. Blood. 2012 May 3;119(18):4115-22. Epub 2012 Mar 6. link to original article contains dosing details in manuscript PubMed

Romidepsin monotherapy

Regimen

Study	Years of enrollment	Evidence	Efficacy
Piekarz et al. 2009 (NIH 01-C-0049)	NR	Phase 2 (RT)	ORR: 34% (95% CI, 23-46)
Whittaker et al. 2010 (GPI-04-0001)	2005-2007	Phase 2 (RT)	ORR: 34% (95% CI, 25-45)

Targeted therapy

Romidepsin (Istodax) 14 mg/m² IV over 4 hours once per day on days 1, 8, 15

28-day cycle for up to 6 cycles; optional extension of treatment for patients with SD or better

References

NIH 01-C-0049: Piekarz RL, Frye R, Turner M, Wright JJ, Allen SL, Kirschbaum MH, Zain J, Prince HM, Leonard JP, Geskin LJ, Reeder C, Joske D, Figg WD, Gardner ER, Steinberg SM, Jaffe ES, Stetler-Stevenson M, Lade S, Fojo AT, Bates SE. Phase II multi-institutional trial of the histone deacetylase inhibitor romidepsin as monotherapy for patients with cutaneous T-cell lymphoma. J Clin Oncol. 2009 Nov 10;27(32):5410-7. Epub 2009 Oct 13. link to original article contains dosing details in manuscript link to PMC article PubMed NCT00020436
GPI-04-0001: Whittaker SJ, Demierre MF, Kim EJ, Rook AH, Lerner A, Duvic M, Scarisbrick J, Reddy S, Robak T, Becker JC, Samtsov A, McCulloch W, Kim YH. Final results from a multicenter, international, pivotal study of romidepsin in refractory cutaneous T-cell lymphoma. J Clin Oncol. 2010 Oct 10;28(29):4485-91. Epub 2010 Aug 9. link to original article contains dosing details in manuscript PubMed NCT00106431

Vorinostat monotherapy

Regimen

FDA-recommended dose

Study	Years of enrollment	Evidence	Comparator	Comparative Efficacy
Duvic et al. 2006	NR	Phase 2a (RT)		ORR: 24%
Olsen et al. 2007 (Merck 0683-001)	NR	Phase 2b (RT)		ORR: 30%
Kim et al. 2018 (MAVORIC)	2012-2016	Phase 3 (C)	Mogamulizumab	Inferior PFS

Note: Duvic et al. 2006 evaluated several different doses, but this is the one that "had the most favorable safety profile". To our knowledge, this regimen was not tested as an experimental arm in an RCT prior to becoming a standard comparator arm.

Targeted therapy

Vorinostat (Zolinza) 400 mg PO once per day

Continued indefinitely

References

Duvic M, Talpur R, Ni X, Zhang C, Hazarika P, Kelly C, Chiao JH, Reilly JF, Ricker JL, Richon VM, Frankel SR. Phase 2 trial of oral vorinostat (suberoylanilide hydroxamic acid, SAHA) for refractory cutaneous T-cell lymphoma (CTCL). Blood. 2007 Jan 1;109(1):31-9. Epub 2006 Sep 7. Erratum in: Blood. 2007 Jun 15;109(12):5086. link to original article link to PMC article contains dosing details in abstract PubMed
Merck 0683-001: Olsen EA, Kim YH, Kuzel TM, Pacheco TR, Foss FM, Parker S, Frankel SR, Chen C, Ricker JL, Arduino JM, Duvic M. Phase IIb multicenter trial of vorinostat in patients with persistent, progressive, or treatment refractory cutaneous T-cell lymphoma. J Clin Oncol. 2007 Jul 20;25(21):3109-15. link to original article contains dosing details in manuscript PubMed NCT00091559
1. Update: Duvic M, Olsen EA, Breneman D, Pacheco TR, Parker S, Vonderheid EC, Abuav R, Ricker JL, Rizvi S, Chen C, Boileau K, Gunchenko A, Sanz-Rodriguez C, Geskin LJ. Evaluation of the long-term tolerability and clinical benefit of vorinostat in patients with advanced cutaneous T-cell lymphoma. Clin Lymphoma Myeloma. 2009 Dec;9(6):412-6. link to original article PubMed
MAVORIC: Kim YH, Bagot M, Pinter-Brown L, Rook AH, Porcu P, Horwitz SM, Whittaker S, Tokura Y, Vermeer M, Zinzani PL, Sokol L, Morris S, Kim EJ, Ortiz-Romero PL, Eradat H, Scarisbrick J, Tsianakas A, Elmets C, Dalle S, Fisher DC, Halwani A, Poligone B, Greer J, Fierro MT, Khot A, Moskowitz AJ, Musiek A, Shustov A, Pro B, Geskin LJ, Dwyer K, Moriya J, Leoni M, Humphrey JS, Hudgens S, Grebennik DO, Tobinai K, Duvic M; MAVORIC Investigators. Mogamulizumab versus vorinostat in previously treated cutaneous T-cell lymphoma (MAVORIC): an international, open-label, randomised, controlled phase 3 trial. Lancet Oncol. 2018 Sep;19(9):1192-1204. Epub 2018 Aug 9. link to original article contains dosing details in abstract PubMed NCT01728805

Relapsed or refractory, subsequent lines of treatment

Denileukin diftitox monotherapy

Regimen

Study	Evidence
Duvic et al. 2012	Non-randomized

Chemotherapy

Denileukin diftitox (Ontak) 18 mcg/kg IV once per day on days 1 to 5

21-day cycle for up to 8 cycles

References

Duvic M, Martin AG, Olsen EA, Fivenson DP, Prince HM. Efficacy and safety of denileukin diftitox retreatment in patients with relapsed cutaneous T-cell lymphoma. Leuk Lymphoma. 2013 Mar;54(3):514-9. Epub 2012 Sep 3. link to original article contains dosing details in abstract PubMed

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 *'''2018:''' Willemze et al. [https://www.esmo.org/Guidelines/Haematological-Malignancies/Primary-Cutaneous-Lymphoma Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up]
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 *'''2019:''' Shinohara & Shustov [https://doi.org/10.1182/blood.2019000785 How I treat primary cutaneous CD30 + lymphoproliferative disorders]
@@ Line 27: / Line 25: @@
 *[https://www.nccn.org/professionals/physician_gls/pdf/primary_cutaneous.pdf NCCN Guidelines - Primary Cutaneous Lymphomas]
 *[https://www.nccn.org/professionals/physician_gls/pdf/t-cell.pdf NCCN Guidelines - T-cell Lymphomas]
 =Upfront therapy=
 ==PUVA==
 PUVA: '''<u>P</u>'''soralen & '''<u>U</u>'''ltra-'''<u>V</u>'''iolet '''<u>A</u>'''
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen===
 {| class="wikitable sortable" style="width: 100%; text-align:center;"
@@ Line 54: / Line 51: @@
 |}
 ''Note: to our knowledge, this regimen was not tested as an experimental arm in an RCT in this context, prior to becoming a standard comparator arm.''
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Chemotherapy====
 *[[Methoxsalen (Uvadex)]] (see papers for details)
+</div></div>
 ===References===
 # Edelson R, Berger C, Gasparro F, Jegasothy B, Heald P, Wintroub B, Vonderheid E, Knobler R, Wolff K, Plewig G, McKiernan G, Christiansen I, Oster M, Honigsmann H, Wilford H, Kokoschka E, Rehle T, Perez M, Stingl G, Laroche L. Treatment of cutaneous T-cell lymphoma by extracorporeal photochemotherapy: preliminary results. N Engl J Med. 1987 Feb 5;316(6):297-303. [https://doi.org/10.1056/NEJM198702053160603 link to original article] [https://pubmed.ncbi.nlm.nih.gov/3543674 PubMed]
 # '''EORTC 21011:''' Whittaker S, Ortiz P, Dummer R, Ranki A, Hasan B, Meulemans B, Gellrich S, Knobler R, Stadler R, Karrasch M. 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 Sep;167(3):678-87. [https://doi.org/full/10.1111/j.1365-2133.2012.11156.x link to original article] [https://pubmed.ncbi.nlm.nih.gov/22924950 PubMed] NCT00056056
 ==Topical therapy==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen===
 {| class="wikitable sortable" style="width: 100%; text-align:center;"
@@ Line 79: / Line 76: @@
 |}
 ''Usually consists of high-dose topical steroids or nitrogen mustards; see paper for details.''
+</div></div>
 ===References===
 # Kaye FJ, Bunn PA Jr, Steinberg SM, Stocker JL, Ihde DC, Fischmann AB, Glatstein EJ, Schechter GP, Phelps RM, Foss FM, Parlette HL, Anderson MJ, Sausville EA. A randomized trial comparing combination electron-beam radiation and chemotherapy with topical therapy in the initial treatment of mycosis fungoides. N Engl J Med. 1989 Dec 28;321(26):1784-90. [https://doi.org/10.1056/NEJM198912283212603 link to original article] [https://pubmed.ncbi.nlm.nih.gov/2594037 PubMed]
 =Relapsed or refractory=
 ==Alemtuzumab monotherapy {{#subobject:cd1346|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen {{#subobject:7a3b21|Variant=1}}===
 {| class="wikitable" style="width: 60%; text-align:center;"
@@ Line 98: / Line 93: @@
 |-
 |}
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Targeted therapy====
 *[[Alemtuzumab (Campath)]] 3 mg IV once on day 1, then increased to 10 mg IV once as soon as infusion-related reactions tolerated, then increased to 30 mg IV once as soon as infusion-related reactions tolerated, then 30 mg IV 3 days per week
 '''Up to 12-week course'''
+</div></div>
 ===References===
 # Lundin J, Hagberg H, Repp R, Cavallin-Ståhl E, Fredén S, Juliusson G, Rosenblad E, Tjønnfjord G, Wiklund T, Osterborg A. Phase 2 study of alemtuzumab (anti-CD52 monoclonal antibody) in patients with advanced mycosis fungoides/Sezary syndrome. Blood. 2003 Jun 1;101(11):4267-72. Epub 2003 Jan 23. [http://www.bloodjournal.org/content/101/11/4267.long link to original article] '''contains dosing details in abstract''' [https://pubmed.ncbi.nlm.nih.gov/12543862 PubMed]
 ==Belinostat monotherapy {{#subobject:f8519|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen {{#subobject:693804|Variant=1}}===
 {| class="wikitable" style="width: 60%; text-align:center;"
@@ Line 120: / Line 113: @@
 |-
 |}
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Targeted therapy====
 *[[Belinostat (Beleodaq)]] 1000 mg/m<sup>2</sup> IV over 30 minutes once per day on days 1 to 5
 '''21-day cycles'''
+</div></div>
 ===References===
 <!-- # '''Abstract:''' Pohlman, Brad, Advani, Ranjana, Duvic, Madeleine, Hymes, Kenneth B., Intragumtornchai, Tanin, Lekhakula, Arnuparp, Shpilberg, Ofer, Lerner, Adam, Ben-Yehuda, Dina, beylot-Barry, Marie, Hillen, Uwe, Fagerberg, Jan, Foss, Francine M. Final Results of a Phase II Trial of Belinostat (PXD101) in Patients with Recurrent or Refractory Peripheral or Cutaneous T-Cell Lymphoma. ASH Annual Meeting Abstracts 2009 114: 920. [http://abstracts.hematologylibrary.org/cgi/content/abstract/114/22/920 link to abstract] -->
 # '''PXD101-CLN-6:''' Foss F, Advani R, Duvic M, Hymes KB, Intragumtornchai T, Lekhakula A, Shpilberg O, Lerner A, Belt RJ, Jacobsen ED, Laurent G, Ben-Yehuda D, Beylot-Barry M, Hillen U, Knoblauch P, Bhat G, Chawla S, Allen LF, Pohlman B. A phase II trial of belinostat (PXD101) in patients with relapsed or refractory peripheral or cutaneous T-cell lymphoma. Br J Haematol. 2015 Mar;168(6):811-9. Epub 2014 Nov 17. [https://doi.org/10.1111/bjh.13222 link to original article] '''contains dosing details in abstract''' [https://pubmed.ncbi.nlm.nih.gov/25404094 PubMed] NCT00274651
 ==Bendamustine monotherapy {{#subobject:1a5a99|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen {{#subobject:44f094|Variant=1}}===
 {| class="wikitable" style="width: 60%; text-align:center;"
@@ Line 143: / Line 134: @@
 |-
 |}
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Chemotherapy====
 *[[Bendamustine]] 120 mg/m<sup>2</sup> IV once per day on days 1 & 2
 '''21-day cycle for 6 cycles'''
+</div></div>
 ===References===
 # '''BENTLY:''' Damaj G, Gressin R, Bouabdallah K, Cartron G, Choufi B, Gyan E, Banos A, Jaccard A, Park S, Tournilhac O, Schiano-de Collela JM, Voillat L, Joly B, Le Gouill S, Saad A, Cony-Makhoul P, Vilque JP, Sanhes L, Schmidt-Tanguy A, Bubenheim M, Houot R, Diouf M, Marolleau JP, Béné MC, Martin A, Lamy T. Results from a prospective, open-label, phase II trial of bendamustine in refractory or relapsed T-cell lymphomas: the BENTLY trial. J Clin Oncol. 2013 Jan 1;31(1):104-10. Epub 2012 Oct 29. [https://doi.org/10.1200/jco.2012.43.7285 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/23109692 PubMed] NCT00959686
 ==Bexarotene monotherapy {{#subobject:feb851|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen variant #1, 48 weeks {{#subobject:67ef8a|Variant=1}}===
 {| class="wikitable sortable" style="width: 100%; text-align:center;"
@@ Line 170: / Line 159: @@
 |}
 ''<sup>1</sup>Reported efficacy is based on the 2021 update.''<br>
-''This dose is considered the target dose in ALCANZA.''
+''Note: This dose is considered the target dose in ALCANZA.''
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Targeted therapy====
 *[[Bexarotene (Targretin)]] 300 mg/m<sup>2</sup> PO once per day
 '''48-week course'''
+</div></div><br>
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen variant #2, indefinite {{#subobject:68je8a|Variant=1}}===
 {| class="wikitable sortable" style="width: 100%; text-align:center;"
@@ Line 198: / Line 188: @@
 |}
 ''Note: This dose is considered the optimal starting dose by Duvic et al. 2001a.''
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Targeted therapy====
 *[[Bexarotene (Targretin)]] 300 mg/m<sup>2</sup> PO once per day
 '''Continued indefinitely'''
+</div></div>
 ===References===
 # Duvic M, Hymes K, Heald P, Breneman D, Martin AG, Myskowski P, Crowley C, Yocum RC; Bexarotene Worldwide Study Group. 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 May 1;19(9):2456-71. [https://doi.org/10.1200/jco.2001.19.9.2456 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/11331325 PubMed]
@@ Line 208: / Line 198: @@
 # '''ALCANZA:''' Prince HM, Kim YH, Horwitz SM, Dummer R, Scarisbrick J, Quaglino P, Zinzani PL, Wolter P, Sanches JA, Ortiz-Romero PL, Akilov OE, Geskin L, Trotman J, Taylor K, Dalle S, Weichenthal M, Walewski J, Fisher D, Dréno B, Stadler R, Feldman T, Kuzel TM, Wang Y, Palanca-Wessels MC, Zagadailov E, Trepicchio WL, Zhang W, Lin HM, Liu Y, Huebner D, Little M, Whittaker S, Duvic M; ALCANZA study group. 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 Aug 5;390(10094):555-566. Epub 2017 Jun 7. [https://doi.org/10.1016/S0140-6736(17)31266-7 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/28600132 PubMed] NCT01578499
 ##'''Update:''' Horwitz SM, Scarisbrick JJ, Dummer R, Whittaker S, Duvic M, Kim YH, Quaglino P, Zinzani PL, Bechter O, Eradat H, Pinter-Brown L, Akilov OE, Geskin L, Sanches JA, Ortiz-Romero PL, Weichenthal M, Fisher DC, Walewski J, Trotman J, Taylor K, Dalle S, Stadler R, Lisano J, Bunn V, Little M, Prince HM. Randomized phase 3 ALCANZA study of brentuximab vedotin vs physician's choice in cutaneous T-cell lymphoma: final data. Blood Adv. 2021 Dec 14;5(23):5098-5106. [https://doi.org/10.1182/bloodadvances.2021004710 link to original article] [http://www.ncbi.nlm.nih.gov/pmc/articles/pmc9153035/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/34507350/ PubMed]
 ==Bexarotene & Pralatrexate {{#subobject:8d4e8d|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen {{#subobject:53ada8|Variant=1}}===
 {| class="wikitable sortable" style="width: 80%; text-align:center;"
@@ Line 224: / Line 213: @@
 |-
 |}
-''This dose is the MTD. Note that the abstract contains a typo for bexarotene dosing; the authors have been contacted.''
+''Note: This dose is the MTD. Note that the abstract contains a typo for bexarotene dosing; the authors have been contacted.''
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Targeted therapy====
 *[[Bexarotene (Targretin)]] 150 mg/m<sup>2</sup> PO once per day
 ====Chemotherapy====
 *[[Pralatrexate (Folotyn)]] 15 mg/m<sup>2</sup> IV once per day on days 1, 8, 15
 '''28-day cycles'''
+</div></div>
 ===References===
 # '''PDX-018:''' Duvic M, Kim YH, Zinzani PL, Horwitz SM. Results from a phase I/II open-label, dose-finding study of pralatrexate and oral bexarotene in patients with relapsed/refractory cutaneous T-cell lymphoma. Clin Cancer Res. 2017 Jul;23(14):3552-6. Epub 2017 Feb 6. [http://clincancerres.aacrjournals.org/content/23/14/3552 link to original article] '''contains dosing details in manuscript''' [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5511551/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/28167509 PubMed] NCT01134341
 ==Brentuximab vedotin monotherapy {{#subobject:2781ab|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen {{#subobject:34eb6|Variant=1}}===
 {| class="wikitable sortable" style="width: 100%; text-align:center;"
@@ Line 265: / Line 253: @@
 |}
 ''<sup>1</sup>Reported efficacy is based on the 2021 update.''
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Antibody-drug conjugate therapy====
 *[[Brentuximab vedotin (Adcetris)]] 1.8 mg/kg IV over 30 minutes once on day 1
 '''21-day cycle for up to 16 cycles'''
+</div></div>
 ===References===
 <!-- Presented in part at the 54th Annual Meeting of the American Society of Hematology, December 8-11, 2012, Atlanta, GA, the 56th Annual Meeting of the American Society of Hematology, December 6-9, 2014, San Francisco, CA, and the 72nd Annual Meeting of the Society for Investigative Dermatology, May 9-12, 2012, Raleigh, NC. -->
@@ Line 277: / Line 265: @@
 # '''ALCANZA:''' Prince HM, Kim YH, Horwitz SM, Dummer R, Scarisbrick J, Quaglino P, Zinzani PL, Wolter P, Sanches JA, Ortiz-Romero PL, Akilov OE, Geskin L, Trotman J, Taylor K, Dalle S, Weichenthal M, Walewski J, Fisher D, Dréno B, Stadler R, Feldman T, Kuzel TM, Wang Y, Palanca-Wessels MC, Zagadailov E, Trepicchio WL, Zhang W, Lin HM, Liu Y, Huebner D, Little M, Whittaker S, Duvic M; ALCANZA study group. 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 Aug 5;390(10094):555-566. Epub 2017 Jun 7. [https://doi.org/10.1016/S0140-6736(17)31266-7 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/28600132 PubMed] NCT01578499
 ##'''Update:''' Horwitz SM, Scarisbrick JJ, Dummer R, Whittaker S, Duvic M, Kim YH, Quaglino P, Zinzani PL, Bechter O, Eradat H, Pinter-Brown L, Akilov OE, Geskin L, Sanches JA, Ortiz-Romero PL, Weichenthal M, Fisher DC, Walewski J, Trotman J, Taylor K, Dalle S, Stadler R, Lisano J, Bunn V, Little M, Prince HM. Randomized phase 3 ALCANZA study of brentuximab vedotin vs physician's choice in cutaneous T-cell lymphoma: final data. Blood Adv. 2021 Dec 14;5(23):5098-5106. [https://doi.org/10.1182/bloodadvances.2021004710 link to original article] [http://www.ncbi.nlm.nih.gov/pmc/articles/pmc9153035/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/34507350/ PubMed]
 ==Denileukin diftitox monotherapy {{#subobject:751a63|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen {{#subobject:4e103c|Variant=1}}===
 {| class="wikitable sortable" style="width: 100%; text-align:center;"
@@ Line 306: / Line 292: @@
 |}
 ''Note: Dose is that which was recommended in L4389-11 based on superior response. Up to 3 additional cycles allowed in L4389-10 for patients who had ongoing response.''
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Targeted therapy====
 *[[Denileukin diftitox (Ontak)]] 18 mcg/kg IV over 15 to 60 minutes once per day on days 1 to 5
 ====Supportive therapy====
 *"Premedication with [[Acetaminophen (Tylenol)]] (650 mg in Olsen et al. 2001) and an antihistamine was required 30 to 60 minutes before each infusion" and could be used after infusions as needed.
 *Olsen et al. 2001 used [[Promethazine (Phenergan)]] 25 mg or [[Prochlorperazine (Compazine)]] 10 mg (route/schedule not specified) as needed for nausea.
 *Corticosteroid use was not allowed.
 '''21-day cycles for up to 8 cycles (see note)'''
+</div></div>
 ===References===
 # '''L4389-10:''' Olsen E, Duvic M, Frankel A, Kim Y, Martin A, Vonderheid E, Jegasothy B, Wood G, Gordon M, Heald P, Oseroff A, Pinter-Brown L, Bowen G, Kuzel T, Fivenson D, Foss F, Glode M, Molina A, Knobler E, Stewart S, Cooper K, Stevens S, Craig F, Reuben J, Bacha P, Nichols J. Pivotal phase III trial of two dose levels of denileukin diftitox for the treatment of cutaneous T-cell lymphoma. J Clin Oncol. 2001 Jan 15;19(2):376-88. [https://doi.org/10.1200/jco.2001.19.2.376 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/11208829 PubMed]
@@ Line 322: / Line 307: @@
 <!-- Presented in part at the 46th Annual Meeting of the American Society of Clinical Oncology in Chicago, IL, June 4-8, 2010 -->
 # '''Meta-analysis:''' Duvic M, Geskin L, Prince HM. Duration of response in cutaneous T-cell lymphoma patients treated with denileukin diftitox: results from 3 phase III studies. Clin Lymphoma Myeloma Leuk. 2013 Aug;13(4):377-84. Epub 2013 Jun 14. [http://www.clinical-lymphoma-myeloma-leukemia.com/article/S2152-2650(13)00095-5 link to original article] [https://pubmed.ncbi.nlm.nih.gov/23770157 PubMed]
 ==Lenalidomide monotherapy {{#subobject:d48e4c|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen {{#subobject:2dd505|Variant=1}}===
 {| class="wikitable" style="width: 60%; text-align:center;"
@@ Line 337: / Line 320: @@
 |-
 |}
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Targeted therapy====
 *[[Lenalidomide (Revlimid)]] as follows:
@@ Line 343: / Line 327: @@
 **Cycle 3, if tolerated: 20 mg PO once per day on days 1 to 21
 **Cycle 4 onwards, if tolerated: 25 mg PO once per day on days 1 to 21
 '''28-day cycle for up to 26 cycles (2 years)'''
+</div></div>
 ===References===
 # '''NU 04H5:''' Querfeld C, Rosen ST, Guitart J, Duvic M, Kim YH, Dusza SW, Kuzel TM. Results of an open-label multicenter phase II trial of lenalidomide monotherapy in refractory mycosis fungoides and Sezary syndrome. Blood. 2014 Feb 20;123(8):1159-66. Epub 2013 Dec 11. [http://www.bloodjournal.org/content/123/8/1159.full link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/24335103 PubMed] NCT00466921
 ==Methotrexate monotherapy {{#subobject:0ff378|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen {{#subobject:dfe411|Variant=1}}===
 {| class="wikitable sortable" style="width: 100%; text-align:center;"
@@ Line 368: / Line 350: @@
 ''<sup>1</sup>Reported efficacy is based on the 2021 update.''<br>
 ''Note: to our knowledge, this regimen was not tested as an experimental arm in an RCT in this context, prior to becoming a standard comparator arm.''
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Chemotherapy====
 *[[Methotrexate (MTX)]] 5 to 50 mg PO once per day on days 1, 8, 15, 22
 '''28-day cycle for 12 cycles'''
+</div></div>
 ===References===
 # '''Retrospective:''' 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 Nov;49(5):873-8. [https://www.jaad.org/article/S0190-9622(03)01591-3 link to original article] [https://pubmed.ncbi.nlm.nih.gov/14576667 PubMed]
 # '''ALCANZA:''' Prince HM, Kim YH, Horwitz SM, Dummer R, Scarisbrick J, Quaglino P, Zinzani PL, Wolter P, Sanches JA, Ortiz-Romero PL, Akilov OE, Geskin L, Trotman J, Taylor K, Dalle S, Weichenthal M, Walewski J, Fisher D, Dréno B, Stadler R, Feldman T, Kuzel TM, Wang Y, Palanca-Wessels MC, Zagadailov E, Trepicchio WL, Zhang W, Lin HM, Liu Y, Huebner D, Little M, Whittaker S, Duvic M; ALCANZA study group. 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 Aug 5;390(10094):555-566. Epub 2017 Jun 7. [https://doi.org/10.1016/S0140-6736(17)31266-7 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/28600132 PubMed] NCT01578499
 ##'''Update:''' Horwitz SM, Scarisbrick JJ, Dummer R, Whittaker S, Duvic M, Kim YH, Quaglino P, Zinzani PL, Bechter O, Eradat H, Pinter-Brown L, Akilov OE, Geskin L, Sanches JA, Ortiz-Romero PL, Weichenthal M, Fisher DC, Walewski J, Trotman J, Taylor K, Dalle S, Stadler R, Lisano J, Bunn V, Little M, Prince HM. Randomized phase 3 ALCANZA study of brentuximab vedotin vs physician's choice in cutaneous T-cell lymphoma: final data. Blood Adv. 2021 Dec 14;5(23):5098-5106. [https://doi.org/10.1182/bloodadvances.2021004710 link to original article] [http://www.ncbi.nlm.nih.gov/pmc/articles/pmc9153035/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/34507350/ PubMed]
 ==Mogamulizumab monotherapy {{#subobject:8d8ae3|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen variant #1, 8-week course {{#subobject:2848bf|Variant=1}}===
 {| class="wikitable" style="width: 40%; text-align:center;"
@@ Line 389: / Line 370: @@
 |-
 |}
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Targeted therapy====
 *[[Mogamulizumab (Poteligeo)]] 1 mg/kg IV once per day on days 1, 8, 15, 22
 '''28-day cycle for 2 cycles'''
+</div></div><br>
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen variant #2, indefinite {{#subobject:097b16|Variant=1}}===
 {| class="wikitable" style="color:white; background-color:#404040"
@@ Line 420: / Line 402: @@
 |}
 ''Note: in KW-0761-001, a two-week "period of observation" was undertaken after cycle 1.''
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Targeted therapy====
 *[[Mogamulizumab (Poteligeo)]] as follows:
 **Cycle 1: 1 mg/kg IV once per day on days 1, 8, 15, 22
 **Cycle 2 onwards: 1 mg/kg IV once per day on days 1 & 15
 '''28-day cycles'''
+</div></div>
 ===References===
 # '''KW-0761-004:''' Ogura M, Ishida T, Hatake K, Taniwaki M, Ando K, Tobinai K, Fujimoto K, Yamamoto K, Miyamoto T, Uike N, Tanimoto M, Tsukasaki K, Ishizawa K, Suzumiya J, Inagaki H, Tamura K, Akinaga S, Tomonaga M, Ueda R. Multicenter phase II study of mogamulizumab (KW-0761), a defucosylated anti-cc chemokine receptor 4 antibody, in patients with relapsed peripheral T-cell lymphoma and cutaneous T-cell lymphoma. J Clin Oncol. 2014 Apr 10;32(11):1157-63. Epub 2014 Mar 10. [https://doi.org/10.1200/jco.2013.52.0924 link to original article] '''contains dosing details in abstract''' [https://pubmed.ncbi.nlm.nih.gov/24616310 PubMed] NCT01192984
 # '''KW-0761-001:''' Duvic M, Pinter-Brown LC, Foss FM, Sokol L, Jorgensen JL, Challagundla P, Dwyer KM, Zhang X, Kurman MR, Ballerini R, Liu L, Kim YH. Phase 1/2 study of mogamulizumab, a defucosylated anti-CCR4 antibody, in previously treated patients with cutaneous T-cell lymphoma. Blood. 2015 Mar 19;125(12):1883-9. Epub 2015 Jan 20. [http://www.bloodjournal.org/content/125/12/1883.long link to original article] '''contains dosing details in abstract''' [https://pubmed.ncbi.nlm.nih.gov/25605368 PubMed] NCT00888927
 # '''MAVORIC:''' Kim YH, Bagot M, Pinter-Brown L, Rook AH, Porcu P, Horwitz SM, Whittaker S, Tokura Y, Vermeer M, Zinzani PL, Sokol L, Morris S, Kim EJ, Ortiz-Romero PL, Eradat H, Scarisbrick J, Tsianakas A, Elmets C, Dalle S, Fisher DC, Halwani A, Poligone B, Greer J, Fierro MT, Khot A, Moskowitz AJ, Musiek A, Shustov A, Pro B, Geskin LJ, Dwyer K, Moriya J, Leoni M, Humphrey JS, Hudgens S, Grebennik DO, Tobinai K, Duvic M; MAVORIC Investigators. Mogamulizumab versus vorinostat in previously treated cutaneous T-cell lymphoma (MAVORIC): an international, open-label, randomised, controlled phase 3 trial. Lancet Oncol. 2018 Sep;19(9):1192-1204. Epub 2018 Aug 9. [https://doi.org/10.1016/S1470-2045(18)30379-6 link to original article] '''contains dosing details in abstract''' [https://pubmed.ncbi.nlm.nih.gov/30100375 PubMed] NCT01728805
 ==Pralatrexate monotherapy {{#subobject:1df1c0|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen {{#subobject:ac3b67|Variant=1}}===
 {| class="wikitable" style="width: 60%; text-align:center;"
@@ Line 446: / Line 426: @@
 |-
 |}
-''Dose is that identified as recommended based on de-escalation strategy.''
+''Note: Dose is that identified as recommended based on de-escalation strategy.''
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Chemotherapy====
 *[[Pralatrexate (Folotyn)]] 15 mg/m<sup>2</sup> IV push once per day on days 1, 8, 15
 ====Supportive therapy====
 *[[Cyanocobalamin (Vitamin B12)]] 1 mg IM once every 8 to 10 weeks, within 10 weeks of treatment initiation
 *[[Folic acid (Folate)]] 1 mg PO once per day, starting at least 10 days prior to treatment initiation
 '''28-day cycles'''
+</div></div>
 ===References===
 # Horwitz SM, Kim YH, Foss F, Zain JM, Myskowski PL, Lechowicz MJ, Fisher DC, Shustov AR, Bartlett NL, Delioukina ML, Koutsoukos T, Saunders ME, O'Connor OA, Duvic M. Identification of an active, well-tolerated dose of pralatrexate in patients with relapsed or refractory cutaneous T-cell lymphoma. Blood. 2012 May 3;119(18):4115-22. Epub 2012 Mar 6. [http://www.bloodjournal.org/content/119/18/4115.long link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/22394596 PubMed]
 ==Romidepsin monotherapy {{#subobject:b4c097|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen {{#subobject:7ef1f5|Variant=1}}===
 {| class="wikitable sortable" style="width: 80%; text-align:center;"
@@ Line 480: / Line 457: @@
 |-
 |}
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Targeted therapy====
 *[[Romidepsin (Istodax)]] 14 mg/m<sup>2</sup> IV over 4 hours once per day on days 1, 8, 15
 '''28-day cycle for up to 6 cycles; optional extension of treatment for patients with SD or better'''
+</div></div>
 ===References===
 # '''NIH 01-C-0049:''' Piekarz RL, Frye R, Turner M, Wright JJ, Allen SL, Kirschbaum MH, Zain J, Prince HM, Leonard JP, Geskin LJ, Reeder C, Joske D, Figg WD, Gardner ER, Steinberg SM, Jaffe ES, Stetler-Stevenson M, Lade S, Fojo AT, Bates SE. Phase II multi-institutional trial of the histone deacetylase inhibitor romidepsin as monotherapy for patients with cutaneous T-cell lymphoma. J Clin Oncol. 2009 Nov 10;27(32):5410-7. Epub 2009 Oct 13. [https://doi.org/10.1200/jco.2008.21.6150 link to original article] '''contains dosing details in manuscript''' [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2773225/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/19826128 PubMed] NCT00020436
 # '''GPI-04-0001:''' Whittaker SJ, Demierre MF, Kim EJ, Rook AH, Lerner A, Duvic M, Scarisbrick J, Reddy S, Robak T, Becker JC, Samtsov A, McCulloch W, Kim YH. Final results from a multicenter, international, pivotal study of romidepsin in refractory cutaneous T-cell lymphoma. J Clin Oncol. 2010 Oct 10;28(29):4485-91. Epub 2010 Aug 9. [https://doi.org/10.1200/jco.2010.28.9066 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/20697094 PubMed] NCT00106431
 ==Vorinostat monotherapy {{#subobject:6a3a5b|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen {{#subobject:d18568|Variant=1}}===
 {| class="wikitable" style="color:white; background-color:#404040"
@@ Line 523: / Line 499: @@
 |}
 ''Note: Duvic et al. 2006 evaluated several different doses, but this is the one that "had the most favorable safety profile". To our knowledge, this regimen was not tested as an experimental arm in an RCT prior to becoming a standard comparator arm.''
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Targeted therapy====
 *[[Vorinostat (Zolinza)]] 400 mg PO once per day
 '''Continued indefinitely'''
+</div></div>
 ===References===
 # Duvic M, Talpur R, Ni X, Zhang C, Hazarika P, Kelly C, Chiao JH, Reilly JF, Ricker JL, Richon VM, Frankel SR. Phase 2 trial of oral vorinostat (suberoylanilide hydroxamic acid, SAHA) for refractory cutaneous T-cell lymphoma (CTCL). Blood. 2007 Jan 1;109(1):31-9. Epub 2006 Sep 7. Erratum in: Blood. 2007 Jun 15;109(12):5086. [http://www.bloodjournal.org/content/109/1/31.long link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1785068/ link to PMC article] '''contains dosing details in abstract''' [https://pubmed.ncbi.nlm.nih.gov/16960145 PubMed]
@@ Line 533: / Line 509: @@
 ## '''Update:''' Duvic M, Olsen EA, Breneman D, Pacheco TR, Parker S, Vonderheid EC, Abuav R, Ricker JL, Rizvi S, Chen C, Boileau K, Gunchenko A, Sanz-Rodriguez C, Geskin LJ. Evaluation of the long-term tolerability and clinical benefit of vorinostat in patients with advanced cutaneous T-cell lymphoma. Clin Lymphoma Myeloma. 2009 Dec;9(6):412-6. [https://doi.org/10.3816/clm.2009.n.082 link to original article] [https://pubmed.ncbi.nlm.nih.gov/19951879 PubMed]
 # '''MAVORIC:''' Kim YH, Bagot M, Pinter-Brown L, Rook AH, Porcu P, Horwitz SM, Whittaker S, Tokura Y, Vermeer M, Zinzani PL, Sokol L, Morris S, Kim EJ, Ortiz-Romero PL, Eradat H, Scarisbrick J, Tsianakas A, Elmets C, Dalle S, Fisher DC, Halwani A, Poligone B, Greer J, Fierro MT, Khot A, Moskowitz AJ, Musiek A, Shustov A, Pro B, Geskin LJ, Dwyer K, Moriya J, Leoni M, Humphrey JS, Hudgens S, Grebennik DO, Tobinai K, Duvic M; MAVORIC Investigators. Mogamulizumab versus vorinostat in previously treated cutaneous T-cell lymphoma (MAVORIC): an international, open-label, randomised, controlled phase 3 trial. Lancet Oncol. 2018 Sep;19(9):1192-1204. Epub 2018 Aug 9. [https://doi.org/10.1016/S1470-2045(18)30379-6 link to original article] '''contains dosing details in abstract''' [https://pubmed.ncbi.nlm.nih.gov/30100375 PubMed] NCT01728805
 =Relapsed or refractory, subsequent lines of treatment=
 ==Denileukin diftitox monotherapy {{#subobject:bf44d8|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen {{#subobject:3a3d12|Variant=1}}===
 {| class="wikitable" style="width: 40%; text-align:center;"
@@ Line 546: / Line 521: @@
 |-
 |}
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Chemotherapy====
 *[[Denileukin diftitox (Ontak)]] 18 mcg/kg IV once per day on days 1 to 5
 '''21-day cycle for up to 8 cycles'''
+</div></div>
 ===References===
 # Duvic M, Martin AG, Olsen EA, Fivenson DP, Prince HM. Efficacy and safety of denileukin diftitox retreatment in patients with relapsed cutaneous T-cell lymphoma. Leuk Lymphoma. 2013 Mar;54(3):514-9. Epub 2012 Sep 3. [https://doi.org/10.3109/10428194.2012.720372 link to original article] '''contains dosing details in abstract''' [https://pubmed.ncbi.nlm.nih.gov/22891708 PubMed]
 [[Category:Cutaneous T-cell lymphoma regimens]]
 [[Category:Disease-specific pages]]
 [[Category:Cutaneous lymphomas]]
 [[Category:T-cell lymphomas]]

Difference between revisions of "Cutaneous T-cell lymphoma"

Revision as of 16:12, 15 October 2022

Guidelines

ESMO

"How I Treat"

NCCN

Upfront therapy

PUVA

Regimen

Chemotherapy

References

Topical therapy

Regimen

References

Relapsed or refractory

Alemtuzumab monotherapy

Regimen

Targeted therapy

References

Belinostat monotherapy

Regimen

Targeted therapy

References

Bendamustine monotherapy

Regimen

Chemotherapy

References

Bexarotene monotherapy

Regimen variant #1, 48 weeks

Targeted therapy

Regimen variant #2, indefinite

Targeted therapy

References

Bexarotene & Pralatrexate

Regimen

Targeted therapy

Chemotherapy

References

Brentuximab vedotin monotherapy

Regimen

Antibody-drug conjugate therapy

References

Denileukin diftitox monotherapy

Regimen

Targeted therapy

Supportive therapy

References

Lenalidomide monotherapy

Regimen

Targeted therapy

References

Methotrexate monotherapy

Regimen

Chemotherapy

References

Mogamulizumab monotherapy

Regimen variant #1, 8-week course

Targeted therapy

Regimen variant #2, indefinite

Targeted therapy

References

Pralatrexate monotherapy

Regimen

Chemotherapy

Supportive therapy

References

Romidepsin monotherapy

Regimen

Targeted therapy

References

Vorinostat monotherapy

Regimen

Targeted therapy

References

Relapsed or refractory, subsequent lines of treatment

Denileukin diftitox monotherapy

Regimen

Chemotherapy

References

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