Skip to main content

Pruritus in Cutaneous T-cell Lymphoma

  • Chapter
  • First Online:
Pruritus

Abstract

Cutaneous T-cell lymphomas (CTCL) account for about 80% of skin lymphomas. CTCL encompasses a diverse group of diseases that are characterized by malignant T lymphocytes that initially home to the skin.1 Within the CTCL, mykosis fungoides (MF) is the most common variant.2 The classical form of MF is characterized by erythematous flat patches in early stages, which may progress to palpable reddish-brown infiltrated plaques. In late stages of MF, the patients show a combination of patches, plaques and tumor lesions or may present as generalized diffuse erythema. A prolonged natural history is one of the remarkable clinical features of MF.3 By contrast, Sezary syndrome (SS) is a rare disease characterized by erythroderma, generalized lymphadenopathy and the presence of atypical convoluted Sézary T cells in the blood count.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 129.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 169.00
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Burg G, Kempf W, Cozzio A, et al. WHO/EORTC classification of cutaneous lymphomas 2005: histological and molecular aspects. J Cutan Pathol. 2005;32:647-674.

    Article  PubMed  Google Scholar 

  2. Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105:3768-3785.

    Article  PubMed  CAS  Google Scholar 

  3. Olsen E, Vonderheid E, Pimpinelli N, 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;110:1713-1722.

    Article  PubMed  CAS  Google Scholar 

  4. Kotz EA, Anderson D, Thiers BH. Cutaneous T-cell lymphoma. J Eur Acad Dermatol Venereol. 2003;17:131-137.

    Article  PubMed  CAS  Google Scholar 

  5. van Doorn R, Scheffer E, Willemze R. Follicular mycosis fungoides, a distinct disease entity with or without associated follicular mucinosis: a clinicopathologic and follow-up study of 51 patients. Arch Dermatol. 2002;138:191-198.

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  7. Green SB, Byar DP, Lamberg SI. Prognostic variables in mycosis fungoides. Cancer. 1981;47:2671-2677.

    Article  PubMed  CAS  Google Scholar 

  8. Ikai K, Uchiyama T, Maeda M, Takigawa M. Sezary-like syndrome in a 10-year-old girl with serologic evidence of human T-cell lymphotropic virus type I infection. Arch Dermatol. 1987;123:1351-1355.

    Article  PubMed  CAS  Google Scholar 

  9. Meister L, Duarte AM, Davis J, Perez JL, Schachner LA. Sezary syndrome in an 11-year-old girl. J Am Acad Dermatol. 1993;28:93-95.

    Article  PubMed  CAS  Google Scholar 

  10. Bowen GM, Stevens SR, Dubin HV, Siddiqui J, Cooper KD. Diagnosis of Sezary syndrome in a patient with generalized pruritus based on early molecular study and flow cytometry. J Am Acad Dermatol. 1995;33:678-680.

    Article  PubMed  CAS  Google Scholar 

  11. Ikoma A, Steinhoff M, Stander S, Yosipovitch G, Schmelz M. The neurobiology of itch. Nat Rev Neurosci. 2006;7:535-547.

    Article  PubMed  CAS  Google Scholar 

  12. Pujol RM, Gallardo F, Llistosella E, et al. Invisible mycosis fungoides: a diagnostic challenge. J Am Acad Dermatol. 2002;47:S168-S171.

    Article  Google Scholar 

  13. Pujol RM, Gallardo F, Llistosella E, et al. Invisible mycosis fungoides: a diagnostic challenge. J Am Acad Dermatol. 2000;42:324-328.

    Article  PubMed  CAS  Google Scholar 

  14. Yamamoto T, Katayama I, Nishioka K. Role of mast cell and stem cell factor in hyperpigmented mycosis fungoides. Blood. 1997;90:1338-1340.

    PubMed  CAS  Google Scholar 

  15. Bouwhuis SA, el Azhary RA, Gibson LE, McEvoy MT, Pittelkow MR. Effect of insulin-dependent diabetes mellitus on response to extracorporeal photopheresis in patients with Sezary syndrome. J Am Acad Dermatol. 2002;47:63-67.

    Article  PubMed  Google Scholar 

  16. Duvic M, Hymes K, Heald 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;19:2456-2471.

    PubMed  CAS  Google Scholar 

  17. Duvic M, Kuzel TM, Olsen EA, et al. Quality-of-life improvements in cutaneous T-cell lymphoma patients treated with denileukin diftitox (ONTAK). Clin Lymphoma. 2002;2:222-228.

    Article  PubMed  CAS  Google Scholar 

  18. Lundin J, Hagberg H, Repp R, et al. Phase 2 study of alemtuzumab (anti-CD52 monoclonal antibody) in patients with advanced mycosis fungoides/Sezary syndrome. Blood. 2003;101:4267-4272.

    Article  PubMed  CAS  Google Scholar 

  19. Duvic M, Talpur R, Ni X, et al. Phase 2 trial of oral vorinostat (suberoylanilide hydroxamic acid, SAHA) for refractory cutaneous T-cell lymphoma (CTCL). Blood. 2007;109:31-39.

    Article  PubMed  CAS  Google Scholar 

  20. Mann BS, Johnson JR, He K, et al. Vorinostat for treatment of cutaneous manifestations of advanced primary cutaneous T-cell lymphoma. Clin Cancer Res. 2007;13:2318-2322.

    Article  PubMed  CAS  Google Scholar 

  21. Olsen EA, Kim YH, Kuzel TM, et al. Phase IIb multicenter trial of vorinostat in patients with persistent, progressive, or treatment refractory cutaneous T-cell lymphoma. J Clin Oncol. 2007;25:3109-3115.

    Google Scholar 

  22. Wilson LD, Quiros PA, Kolenik SA, et al. Additional courses of total skin electron beam therapy in the treatment of patients with recurrent cutaneous T-cell lymphoma. J Am Acad Dermatol. 1996;35:69-73.

    Article  PubMed  CAS  Google Scholar 

  23. Lowe MN, Plosker GL. Bexarotene. Am J Clin Dermatol. 2000;1:245-250.

    Article  PubMed  CAS  Google Scholar 

  24. Singh F, Lebwohl MG. Cutaneous T-cell lymphoma treatment using bexarotene and PUVA: a case series. J Am Acad Dermatol. 2004;51:570-573.

    Article  PubMed  Google Scholar 

  25. Georgieva J, Steinhoff M, Orfanos CE, Treudler R. Ethylene-oxide-induced pruritus associated with extracorporeal photochemotherapy. Transfusion. 2004;44:1532-1533.

    Article  PubMed  Google Scholar 

  26. Tran D, Kwok YK, Goh CL. A retrospective review of PUVA therapy at the National Skin Centre of Singapore. Photodermatol Photoimmunol Photomed. 2001;17:164-67.

    Article  PubMed  CAS  Google Scholar 

  27. Demierre MF, Taverna J. Mirtazapine and gabapentin for reducing pruritus in cutaneous T-cell lymphoma. J Am Acad Dermatol. 2006;55:543-544.

    Article  PubMed  Google Scholar 

  28. el Azhary RA, Bouwhuis SA. Oral bexarotene in a therapy-resistant Sezary syndrome patient: observations on Sezary cell compartmentalization. Int J Dermatol. 2005;44:25-28.

    Article  PubMed  Google Scholar 

  29. Lowe NJ, Cripps DJ, Dufton PA, Vickers CF. Photochemo-therapy for mycosis fungoides: a clinical and histological study. Arch Dermatol. 1979;115:50-53.

    Article  PubMed  CAS  Google Scholar 

  30. Kessler JF, Meyskens FL, Jr., Levine N, Lynch PJ, Jones SE. Treatment of cutaneous T-cell lymphoma (mycosis fungoides) with 13-cis-retinoic acid. Lancet. 1983;1:1345-1347.

    Article  PubMed  CAS  Google Scholar 

  31. Esteve E, Bagot M, Joly P, et al. A prospective study of cutaneous intolerance to topical mechlorethamine therapy in patients with cutaneous T-cell lymphomas. French Study Group of Cutaneous Lymphomas. Arch Dermatol. 1999;135:1349-1353.

    Article  PubMed  CAS  Google Scholar 

  32. Heald P, Mehlmauer M, Martin AG, Crowley CA, Yocum RC, Reich SD. Topical bexarotene therapy for patients with refractory or persistent early-stage cutaneous T-cell lymphoma: results of the phase III clinical trial. J Am Acad Dermatol. 2003;49:801-815.

    Article  PubMed  Google Scholar 

  33. Dumontet C, Thomas L, Berard F, Gimonet JF, Coiffier B. A phase II trial of miltefosine in patients with cutaneous T-cell lymphoma. Bull Cancer. 2006;93:E115-E118.

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2010 Springer-Verlag London Limited

About this chapter

Cite this chapter

Görge, T., Schiller, M. (2010). Pruritus in Cutaneous T-cell Lymphoma. In: Misery, L., Ständer, S. (eds) Pruritus. Springer, London. https://doi.org/10.1007/978-1-84882-322-8_18

Download citation

  • DOI: https://doi.org/10.1007/978-1-84882-322-8_18

  • Published:

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-84882-321-1

  • Online ISBN: 978-1-84882-322-8

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics