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Special Considerations in the Treatment of Mycosis Fungoides

  • Therapy in Practice
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Abstract

Mycosis fungoides is the most common form of cutaneous T cell lymphoma. Although normally presenting to physicians at an early stage and with an indolent course, mycosis fungoides can have a varied presentation. The National Comprehensive Cancer Network (NCCN) has created guidelines for the treatment and staging of mycosis fungoides. Although comprehensive, in practice these guidelines do not provide specific treatment regimens for lesions located in difficult locations and those recalcitrant to the recommended therapy. Because of this, suggestions based on the practices and decisions made at the multidisciplinary cutaneous lymphoma clinic at the Sidney Kimmel Cancer Center at Thomas Jefferson University, Philadelphia, PA, USA, are presented here. Lesions located in areas such as the face and intertriginous zones are often challenging to treat because first-line therapies are often inappropriate, with the locations increasing the possibility of side effects. Additionally, lesions located in the bathing suit distribution are often nonresponsive to first-line therapies for reasons still undetermined. Finally, although well-described, erythroderma secondary to mycosis fungoides is challenging to treat, with controversy surrounding various methods of control. This article both highlights difficult clinical scenarios and reviews the recommended treatment as provided by the NCCN guidelines and provides alternative therapy for lesions that are either difficult to treat because of the location or are recalcitrant to the recommended therapy. With suggestions for the apparent gaps in guidelines, providers can better treat patients who present with more complicated conditions.

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References

  1. National Comprehensive Cancer Network. Mycosis Fungoides/Sezary Syndrome. (Version 4.2018). https://www.nccn.org/professionals/physician_gls/pdf/t-cell.pdf. Accessed 3 July 2018.

  2. Wilcox RA. Cutaneous T-cell lymphoma: 2016 update on diagnosis, risk-stratification, and management. Am J Hematol. 2016;91(1):151–65. https://doi.org/10.1002/ajh.24233.

    Article  CAS  PubMed  Google Scholar 

  3. Ahmad K, Rogers S, McNicholas PD, Collins P. Narrowband UVB and PUVA in the treatment of mycosis fungoides: a retrospective study. Acta Derm Venereol. 2007;87(5):413–7.

    Article  CAS  PubMed  Google Scholar 

  4. Clobetasol. Lexi-Drugs. Lexicomp. Wolters Kluwer Health, Inc. Riverwoods. http://online.lexi.com. Accessed 28 July 2018.

  5. Coondoo A, Phiske M, Verma S, Lahiri K. Side-effects of topical steroids: along overdue revisit. Indian Dermatol Online J. 2014;5(4):416–25. https://doi.org/10.4103/2229-5178.142483.

    Article  PubMed  PubMed Central  Google Scholar 

  6. Valchlor [package insert]. Malvern, PA: Ceptaris Therapeutics, Inc.; 2013.

  7. Targretin [package insert]. San Diego: Ligand Pharmaceuticals Inc.

  8. Gül U, Soylu S, Aslan E, Yazar Z, Demiriz M. Uncommon presentation of mycosis fungoides: eyelid margin involvement. J Dermatol. 2008;35(9):581–4. https://doi.org/10.1111/j.13468138.2008.00526.

    Article  PubMed  Google Scholar 

  9. Metin A, Dilek N, Demirseven DD. Fungal infections of the folds (intertriginous areas). Clin Dermatol. 2015;33(4):437–47. https://doi.org/10.1016/j.clindermatol.

    Article  PubMed  Google Scholar 

  10. Martin SJ, Duvic M. Prevalence and treatment of palmoplantar keratoderma and tinea pedis in patients with Sézary syndrome. Int J Dermatol. 2012;51(10):1195–8. https://doi.org/10.1111/j.1365-4632.2011.05204.

    Article  PubMed  Google Scholar 

  11. Olsen EA, Hodak E, Anderson T, Carter JB, Henderson M, Cooper K, Lim HW. Guidelines for phototherapy of mycosis fungoides and Sézary syndrome: a consensus statement of the United States Cutaneous Lymphoma Consortium. J Am Acad Dermatol. 2016;74(1):27–58. https://doi.org/10.1016/j.jaad.2015.09.033.

    Article  PubMed  Google Scholar 

  12. Chan DV, Aneja S, Honda K, Carlson S, Yao M, Katcher J, Cooper KD. Radiation therapy in the management of unilesional primary cutaneous T-cell lymphomas. Br J Dermatol. 2012;166(5):1134–7. https://doi.org/10.1111/j.1365-2133.2011.10728.

    Article  CAS  PubMed  Google Scholar 

  13. Wilson LD, Kacinski BM, Jones GW. Local superficial radiotherapy in the management of minimal stage IA cutaneous T-cell lymphoma (Mycosis Fungoides). Int J Radiat Oncol Biol Phys. 1998;40(1):109–15.

    Article  CAS  PubMed  Google Scholar 

  14. Piccinno R, Caccialanza M, Percivalle S. Minimal stage IA mycosis fungoides. Results of radiotherapy in 15 patients. J Dermatolog Treat. 2009;20(3):165–8. https://doi.org/10.1080/09546630802516571.

  15. Cotter GW, Baglan RJ, Wasserman TH, Mill W. Palliative radiation treatment of cutaneous mycosis fungoides–a dose response. Int J Radiat Oncol Biol Phys. 1983;9(10):1477–80.

    Article  CAS  PubMed  Google Scholar 

  16. Neelis KJ, Schimmel EC, Vermeer MH, Senff NJ, Willemze R, Noordijk EM. Low-dose palliative radiotherapy for cutaneous B- and T-cell lymphomas. Int J Radiat Oncol Biol Phys. 2009;74(1):154–8. https://doi.org/10.1016/j.ijrobp.2008.06.1918.

    Article  PubMed  Google Scholar 

  17. Thomas TO, Agrawal P, Guitart J, Rosen ST, Rademaker AW, Querfeld C, Hayes JP, Kuzel TM, Mittal BB. Outcome of patients treated with a single-fraction dose of palliative radiation for cutaneous T-cell lymphoma. Int J Radiat Oncol Biol Phys. 2013;85(3):747–53. https://doi.org/10.1016/j.ijrobp.2012.05.034.

    Article  PubMed  Google Scholar 

  18. Lichter MD, Karagas MR, Mott LA, Spencer SK, Stukel TA, Greenberg ER. Therapeutic ionizing radiation and the incidence of basal cell carcinoma and squamous cell carcinoma. The New Hampshire Skin Cancer Study Group. Arch Dermatol. 2000 Aug;136(8):1007-11.

  19. Akhyani M, Ghodsi ZS, Toosi S, Dabbaghian H. Erythroderma: a clinical study of 97 cases. BMC Dermatol. 2005;9(5):5.

    Article  Google Scholar 

  20. César A, Cruz M, Mota A, Azevedo F. Erythroderma. A clinical and etiological study of 103 patients. J Dermatol Case Rep. 2016 Mar 31;10(1):1-9. https://doi.org/10.3315/jdcr.2016.1222.

  21. Talpur R, Bassett R, Duvic M. Prevalence and treatment of Staphylococcus aureus colonization in patients with mycosis fungoides and Sézary syndrome. Br J Dermatol. 2008;159(1):105–12. https://doi.org/10.1111/j.1365-2133.2008.08612.

    Article  CAS  PubMed  Google Scholar 

  22. Hoppe RT, Harrison C, Tavallaee M, Bashey S, Sundram U, Li S, Million L, Dabaja B, Gangar P, Duvic M, Kim YH. Low-dose total skin electron beam therapy as an effective modality to reduce disease burden in patients with mycosis fungoides: results of a pooled analysis from 3 phase-II clinical trials. J Am Acad Dermatol. 2015;72(2):286–92. https://doi.org/10.1016/j.jaad.2014.10.014.

    Article  PubMed  Google Scholar 

  23. Querfeld C, Nagelli LV, Rosen ST, Kuzel TM, Guitart J. Bexarotene in the treatment of cutaneous T-cell lymphoma. Expert Opin Pharmacother. 2006;7(7):907–15.

    Article  CAS  PubMed  Google Scholar 

  24. Strander H. Interferon treatment of human neoplasia. Adv Cancer Res. 1986;46:1–265.

    Article  CAS  PubMed  Google Scholar 

  25. Isaacs A, Lindenmann J. Virus interference. I. the interferon. Proc R Soc Lond B Biol Sci 1957;147:258–67.

  26. Roth MS, Foon KA. Alpha interferon in the treatment of hematologic malignancies. Am J Med. 1986;81(5):871–82.

    Article  CAS  PubMed  Google Scholar 

  27. Ross C, Tingsgaard P, Jorgensen H, et al. Interferon treatment of cutaneous T-cell lymphoma. Eur J Haematol. 1993;51(2):63–72.

    Article  CAS  PubMed  Google Scholar 

  28. Vowels BR, Cassin M, Vonderheid EC, et al. Aberrant cytokine production by Sezary syndrome patients:cytokine secretion pattern resembles murine TH2 cells. J Invest Dermatol. 1992;99(1):90–4.

    Article  CAS  PubMed  Google Scholar 

  29. Vowels BR, Lessin SR, Cassin M, et al. Th2 cytokine mRNA expression in skin in cutaneous T-cell lymphoma. J Invest Dermatol. 1994;103(5):669–73.

    Article  CAS  PubMed  Google Scholar 

  30. Asadullah K, Do¨cke WD, Haeuler A, et al. Progression of mycosis fungoides is associated with increasing cutaneous expression of interleukin-10 mRNA. J Invest Dermatol 1996;107(6):833–7.

  31. French LE, Huard B, Wysocka M, et al. Impaired CD40L signaling is a cause of defective IL-12 and TNF-a production in Se´zary syndrome: circumvention by hexameric soluble CD40L. Blood. 2005;105(1):219–25.

    Article  CAS  PubMed  Google Scholar 

  32. Yoo EK, Cassin M, Lessin SR, et al. Complete molecular remission during biologic response modifier therapy for Se´zary syndrome is associated with enhanced helper T type 1 cytokine production and natural killer cell activity. J Am Acad Dermatol. 2001;45(2):208–16.

    Article  CAS  PubMed  Google Scholar 

  33. Furudate S, Fujimura T, Kakizaki A, et al. Tumor-associated M2 macrophages in mycosis fungoides acquire immunomodulatory function by interferon alpha and interferon gamma. J Dermatol Sci. 2016;83(3):182–9.

    Article  CAS  PubMed  Google Scholar 

  34. Suchin KR, Cassin M, Gottleib SL, et al. Increased interleukin 5 production in eosinophilic Se´zary syndrome: regulation by interferon alfa and interleukin 12. J Am Acad Dermatol. 2001;44(1):28–32.

    Article  CAS  PubMed  Google Scholar 

  35. Olsen EA, Rook AH, Zic J, et al. Sézary 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(2):352–404.

    Article  PubMed  Google Scholar 

  36. Koeller JM. Biologic response modifiers: the interferon alfa experience. Am J Hosp Pharm. 1989;46(11 SUPPL. 2):S11–5.

    CAS  PubMed  Google Scholar 

  37. Actimmue [package insert]. Roswell: Vidara Therapeutics; 2013.

  38. Olsen EA. Interferon in the treatment of cutaneous Tcell lymphoma. Dermatol Ther. 2003;16(4):311–21.

    Article  PubMed  Google Scholar 

  39. Hughes CF, Khot A, McCormack C, et al. Lack of durable disease control with chemotherapy for mycosis fungoides and Sezary syndrome: a comparative study of systemic therapy. Blood. 2015;125:71–81.

    Article  CAS  PubMed  Google Scholar 

  40. Suchin KR, Cucchiara AJ, Gottleib SL, et al. Treatment of cutaneous T-cell lymphoma with combined immunomodulatory therapy: a 14-year experience at a single institution. Arch Dermatol. 2002;138:1054–60.

    PubMed  Google Scholar 

  41. Sugaya M, Tokura Y, Hamada T, et al. Phase II study of i.v. interferon-gamma in Japanese patients with mycosis fungoides. J Dermatol 2014;41(1):50–6.

  42. Straus DJ, Duvic M, Kuzel T, et al. Results of a phase II trial of oral bexarotene (Targretin) combined with interferon alfa-2b (Intron-A) for patients with cutaneous T-cell lymphoma. Cancer. 2007;109(9):1799–803.

    Article  CAS  PubMed  Google Scholar 

  43. Rook AH, Prystowsky MB, Cassin M, et al. Combined therapy for Sézary syndrome with extracorporeal photo chemotherapy and low-dose interferon alfa therapy. Clinical, molecular, and immunologic observations. Arch Dermatol. 1991;127(10):1535–40.

  44. Kuzel TM, Roenigk HH Jr, Samuelson E, et al. Effectiveness of interferon alfa-2a combined with phototherapy for mycosis fungoides and the Sézary syndrome. J Clin Oncol. 1995;13(1):257–63.

    Article  CAS  PubMed  Google Scholar 

  45. Garcia-Vega Y, et al. Pharmacokinetic and pharmacodynamics characterization of a new formulation containing synergistic proportions of interferons alpha-2b and gamma (HeberPAG) in patients with mycosis fungoides: an open-label trial. BMC Pharmacol Toxicol. 2012;13:20.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

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    Authors and Affiliations

    1. Department of Dermatology and Cutaneous Biology, Sidney Kimmel College at Jefferson University Hospital, Thomas Jefferson University, 833 Chestnut Street, Suite 740, Philadelphia, PA, 19107, USA

      Robert Duffy, Tara Jennings & Joya Sahu

    2. Department of Radiation Oncology, Sidney Kimmel College at Jefferson University Hospital, Philadelphia, PA, USA

      Andrew Song & Wenyin Shi

    3. Department of Medical Oncology, Sidney Kimmel College at Jefferson University Hospital, Philadelphia, PA, USA

      Saritha Kartan, Pierluigi Porcu & Onder Alpdogan

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    1. Robert Duffy
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    Correspondence to Joya Sahu.

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    Robert Duffy, Tara Jennings, Saritha Kartan, Andrew Song, Wenyin Shi, Pierluigi Porcu, Onder Alpdogan, and Joya Sahu have no conflicts of interest that might be relevant to the contents of this manuscript.

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    Robert Duffy and Tara Jennings contributed equally to this work.

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    Duffy, R., Jennings, T., Kartan, S. et al. Special Considerations in the Treatment of Mycosis Fungoides. Am J Clin Dermatol 20, 571–578 (2019). https://doi.org/10.1007/s40257-019-00431-z

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