Advertisement

Primary Cutaneous Lymphomas

  • Wolfgang Bauer
  • Robert KnoblerEmail author

Abstract

Although being rare diseases, cutaneous lymphomas, especially mycosis fungoides and Sézary syndrome, represent important differential diagnosis in dermatology that must not be overlooked. Most of the diverse cutaneous lymphomas have, in contrast to nodal lymphomas, a relatively benign clinical course, and treatment should be chosen carefully and in hindsight to this fact.

Keywords

CTCL CBCL Therapy 

Abbreviations

CR

Complete response

EORTC

European Organisation for Research and Treatment of Cancer

ISCL

International Society for Cutaneous Lymphomas

OR

Overall response

ORR

Overall response rate

R-CHOP

Rituximab cyclophosphamide, hydroxydaunorubicin, vincristine, prednisolone

R-COP

Rituximab cyclophosphamide, vincristine, prednisolone

TCR

T-cell receptor gene rearrangement

Reference

  1. Trautinger F. Phototherapy of mycosis fungoides. Photodermatol Photoimmunol Photomed. 2011;27(2):68–74.PubMedGoogle Scholar

Further Reading

  1. Apisarnthanarax N, Talpur R, Ward S, Ni X, Kim HW, Duvic M. Tazarotene 0.1% gel for refractory mycosis fungoides lesions: an open-label pilot study. J Am Acad Dermatol. 2004;50(4):600–7.PubMedGoogle Scholar
  2. Bekkenk MW, Geelen FA, van Voorst Vader PC, Heule F, Geerts ML, van Vloten WA, et al. Primary and secondary cutaneous CD30(+) lymphoproliferative disorders: a report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment. Blood. 2000;95(12):3653–61.PubMedGoogle Scholar
  3. 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(6):784–94.PubMedGoogle Scholar
  4. Campo E, Swerdlow SH, Harris NL, Pileri S, Stein H, Jaffe ES. The 2008 WHO classification of lymphoid neoplasms and beyond: evolving concepts and practical applications. Blood. 2010;117(19):5019–32.Google Scholar
  5. Dani T, Knobler R. Extracorporeal photoimmunotherapy-photopheresis. Front Biosci (Landmark Ed). 2009;14:4769–77.Google Scholar
  6. 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(33):4091–7.PubMedGoogle Scholar
  7. Duvic M, Hymes K, Heald P, Breneman D, Martin AG, 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(9):2456–71.PubMedGoogle Scholar
  8. Duvic M, Reddy SA, Pinter-Brown L, Korman NJ, Zic J, Kennedy DA, et al. A phase II study of SGN-30 in cutaneous anaplastic large cell lymphoma and related lymphoproliferative disorders. Clin Cancer Res. 2009;15(19):6217–24.PubMedGoogle Scholar
  9. Duvic M, Donato M, Dabaja B, Richmond H, Singh L, Wei W, et al. Total skin electron beam and non-myeloablative allogeneic hematopoietic stem-cell transplantation in advanced mycosis fungoides and Sezary syndrome. J Clin Oncol. 2010;28(14):2365–72.PubMedGoogle Scholar
  10. Edelson R, Berger C, Gasparro F, Jegasothy B, Heald P, Wintroub B, et al. Treatment of cutaneous T-cell lymphoma by extracorporeal photochemotherapy. Preliminary results. N Engl J Med. 1987;316(6):297–303.PubMedGoogle Scholar
  11. Ellis L, Pan Y, Smyth GK, George DJ, McCormack C, Williams-Truax R, et al. Histone deacetylase inhibitor panobinostat induces clinical responses with associated alterations in gene expression profiles in cutaneous T-cell lymphoma. Clin Cancer Res. 2008;14(14):4500–10.PubMedGoogle Scholar
  12. Evans PA, Pott C, Groenen PJ, Salles G, Davi F, Berger F, et al. Significantly improved PCR-based clonality testing in B-cell malignancies by use of multiple immunoglobulin gene targets. Report of the BIOMED-2 Concerted Action BHM4-CT98-3936. Leukemia. 2007;21(2):207–14.PubMedGoogle Scholar
  13. Fujita H, Nagatani T, Miyazawa M, Wada H, Koiwa K, Komatsu H, et al. Primary cutaneous anaplastic large cell lymphoma successfully treated with low-dose oral methotrexate. Eur J Dermatol. 2008;18(3):360–1.PubMedGoogle Scholar
  14. Horwitz SM, Kim YH, Foss F, Zain JM, Myskowski PL, Lechowicz MJ. Identification of an active, well-tolerated dose of pralatrexate in patients with relapsed or refractory cutaneous T-cell lymphoma. Blood. 2012;119(18):4115–22.PubMedGoogle Scholar
  15. Imam MH, Shenoy PJ, Flowers CR, Phillips A, Lechowicz MJ. Incidence and survival patterns of cutaneous T-cell lymphomas in the United States. Leuk Lymphoma. 2013;54(4):752–9.PubMedGoogle Scholar
  16. Kadin ME. Current management of primary cutaneous CD30+ T-cell lymphoproliferative disorders. Oncology (Williston Park). 2009;23(13):1158–64.Google Scholar
  17. Kaye FJ, Bunn Jr PA, Steinberg SM, Stocker JL, Ihde DC, Fischmann AB, et al. 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;321(26):1784–90.PubMedGoogle Scholar
  18. Kempf W, Pfaltz K, Vermeer MH, Cozzio A, Ortiz-Romero PL, Bagot M, et al. EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. Blood. 2011;118(15):4024–35.PubMedPubMedCentralGoogle Scholar
  19. Kennedy GA, Seymour JF, Wolf M, Januszewicz H, Davison J, McCormack C, et al. Treatment of patients with advanced mycosis fungoides and Sezary syndrome with alemtuzumab. Eur J Haematol. 2003;71(4):250–6.PubMedGoogle Scholar
  20. Kim YH, Martinez G, Varghese A, Hoppe RT. Topical nitrogen mustard in the management of mycosis fungoides: update of the Stanford experience. Arch Dermatol. 2003;139(2):165–73.PubMedGoogle Scholar
  21. Kim YH, Willemze R, Pimpinelli N, Whittaker S, Olsen EA, Ranki A, et al. TNM classification system for primary cutaneous lymphomas other than mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the Cutaneous Lymphoma Task Force of the European Organization of Research and Treatment of Cancer (EORTC). Blood. 2007a;110(2):479–84.Google Scholar
  22. Kim YH, Duvic M, Obitz E, Gniadecki R, Iversen L, Osterborg A, et al. Clinical efficacy of zanolimumab (HuMax-CD4): two phase 2 studies in refractory cutaneous T-cell lymphoma. Blood. 2007b;109(11):4655–62.Google Scholar
  23. Knobler R, Berlin G, Calzavara-Pinton P, Greinix H, Jaksch P, Laroche L, et al. Guidelines on the use of extracorporeal photopheresis. J Eur Acad Dermatol Venereol. 2014;28 Suppl 1:1–37.PubMedGoogle Scholar
  24. Kodama K, Massone C, Chott A, Metze D, Kerl H, Cerroni L. Primary cutaneous large B-cell lymphomas: clinicopathologic features, classification, and prognostic factors in a large series of patients. Blood. 2005;106(7):2491–7.PubMedGoogle Scholar
  25. Lessin SR, Duvic M, Guitart J, Pandya AG, Strober BE, Olsen EA, et al. Topical chemotherapy in cutaneous T-cell lymphoma: positive results of a randomized, controlled, multicenter trial testing the efficacy and safety of a novel mechlorethamine, 0.02%, gel in mycosis fungoides. JAMA Dermatol. 2013;149(1):25–32.PubMedPubMedCentralGoogle Scholar
  26. Martinez-Gonzalez MC, Verea-Hernando MM, Yebra-Pimentel MT, Del Pozo J, Mazaira M, Fonseca E. Imiquimod in mycosis fungoides. Eur J Dermatol. 2008;18(2):148–52.PubMedGoogle Scholar
  27. Olsen E, Vonderheid E, Pimpinelli N, Willemze R, Kim Y, Knobler R, et al. Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC). Blood. 2007;110(6):1713–22.PubMedGoogle Scholar
  28. 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(2):352–404.PubMedGoogle Scholar
  29. Olsen EA, Whittaker S, Kim YH, Duvic M, Prince HM, Lessin SR, et al. Clinical end points and response criteria in mycosis fungoides and Sezary syndrome: a consensus statement of the International Society for Cutaneous Lymphomas, the United States Cutaneous Lymphoma Consortium, and the Cutaneous Lymphoma Task Force of the European Organisation for Research and Treatment of Cancer. J Clin Oncol. 2011;29(18):2598–607.PubMedPubMedCentralGoogle Scholar
  30. Paralkar VR, Nasta SD, Morrissey K, Smith J, Vassilev P, Martin ME, et al. Allogeneic hematopoietic SCT for primary cutaneous T cell lymphomas. Bone Marrow Transplant. 2012;47(7):940–5.PubMedGoogle Scholar
  31. Piekarz RL, Frye R, Turner M, Wright JJ, Allen SL, Kirschbaum MH, et al. Phase II multi-institutional trial of the histone deacetylase inhibitor romidepsin as monotherapy for patients with cutaneous T-cell lymphoma. J Clin Oncol. 2009;27(32):5410–7.PubMedPubMedCentralGoogle Scholar
  32. Prince HM, Whittaker S, Hoppe RT. How I treat mycosis fungoides and Sezary syndrome. Blood. 2009;114(20):4337–53.PubMedGoogle Scholar
  33. Querfeld C, Kuzel TM, Kim YH, Porcu P, Duvic M, Musiek A, et al. Multicenter phase II trial of enzastaurin in patients with relapsed or refractory advanced cutaneous T-cell lymphoma. Leuk Lymphoma. 2011;52(8):1474–80.PubMedGoogle Scholar
  34. Querfeld C, Rosen ST, Guitart J, Duvic M, Kim YH, Dusza SW, et al. Results of an open-label multicenter phase II trial of lenalidomide monotherapy in refractory mycosis fungoides and Sezary syndrome. Blood. 2014;123:1159–66.PubMedGoogle Scholar
  35. Russell-Jones R. Extracorporeal photopheresis in cutaneous T-cell lymphoma. Inconsistent data underline the need for randomized studies. Br J Dermatol. 2000;142(1):16–21.PubMedGoogle Scholar
  36. Scarisbrick JJ, Taylor P, Holtick U, Makar Y, Douglas K, Berlin G, et al. U.K. consensus statement on the use of extracorporeal photopheresis for treatment of cutaneous T-cell lymphoma and chronic graft-versus-host disease. Br J Dermatol. 2008;158(4):659–78.PubMedGoogle Scholar
  37. Senff NJ, Hoefnagel JJ, Jansen PM, Vermeer MH, van Baarlen J, Blokx WA, et al. Reclassification of 300 primary cutaneous B-Cell lymphomas according to the new WHO-EORTC classification for cutaneous lymphomas: comparison with previous classifications and identification of prognostic markers. J Clin Oncol. 2007;25(12):1581–7.PubMedGoogle Scholar
  38. Senff NJ, Kluin-Nelemans HC, Willemze R. Results of bone marrow examination in 275 patients with histological features that suggest an indolent type of cutaneous B-cell lymphoma. Br J Haematol. 2008;142(1):52–6.PubMedGoogle Scholar
  39. Senff NJ, Noordijk EM, Kim YH, Bagot M, Berti E, Cerroni L, et al. European Organization for Research and Treatment of Cancer and International Society for Cutaneous Lymphoma consensus recommendations for the management of cutaneous B-cell lymphomas. Blood. 2008;112(5):1600–9.PubMedGoogle Scholar
  40. Stadler R, Assaf C, Klemke CD, Nashan D, Weichenthal M, Dummer R, et al. Brief S2k guidelines – cutaneous lymphomas. J Dtsch Dermatol Ges. 2013;11 Suppl 3:19–28, 0–30.PubMedGoogle Scholar
  41. Suarez AL, Pulitzer M, Horwitz S, Moskowitz A, Querfeld C, Myskowski PL. Primary cutaneous B-cell lymphomas: part I. Clinical features, diagnosis, and classification. J Am Acad Dermatol. 2013;69(3):329 e1–13; quiz 341–2.Google Scholar
  42. Suarez AL, Querfeld C, Horwitz S, Pulitzer M, Moskowitz A, Myskowski PL. Primary cutaneous B-cell lymphomas: part II. Therapy and future directions. J Am Acad Dermatol. 2013;69(3):343 e1–11; quiz 355–6.Google Scholar
  43. Trautinger F, Knobler R, Willemze R, Peris K, Stadler R, Laroche L, et al. EORTC consensus recommendations for the treatment of mycosis fungoides/Sezary syndrome. Eur J Cancer. 2006;42(8):1014–30.PubMedGoogle Scholar
  44. van Krieken JH, Langerak AW, Macintyre EA, Kneba M, Hodges E, Sanz RG, et al. Improved reliability of lymphoma diagnostics via PCR-based clonality testing: report of the BIOMED-2 concerted action BHM4-CT98-3936. Leukemia. 2007;21(2):201–6.PubMedGoogle Scholar
  45. Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105(10):3768–85.PubMedGoogle Scholar
  46. 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.PubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Department of DermatologyVienna General Hospital, AKH, Medical University of ViennaViennaAustria

Personalised recommendations