Abstract
An 83-year-old female presented with a progressing pruritic cutaneous rash that started 8 years ago. On clinical exam there were numerous coalescing, infiltrated, scaly, and partially crusted erythematous plaques distributed over her trunk and extremities and a large fungating ulcerated nodule on her right thigh covering 75% of her total body surface area (Figure 10.1). Lymphoma-associated alopecia and a left axillary lymphadenopathy were also noted. For the past 3–4 months she reported fatigue, severe pruritus, night sweats, 20 pounds of weight loss, and loss of appetite. She was diagnosed with mycosis fungoides (cutaneous patches and plaques) 4 years ago per skin biopsy. Work-up included computed tomography (CT) scans and laboratory tests such as flow cytometry for Sézary cells that were all negative for systemic involvement. She was initially treated with psoralen and UV-A phototherapy (PUVA) thrice weekly and/or topical rexinoid inhibitor (bexarotene 1% gel) daily for about 2 years, but discontinued due to development of cutaneous ulcerated tumors and lymphadenopathy. Subsequent regimens included vorinostat and prednisone. She developed intractable pruritus and worsening skin rash for the past 3 months for which she was recently started on topical nitrogen mustard 0.016% gel and oral prednisone (20 mg). Her past clinical history is remarkable for lung adenocarcinoma treated with partial lobectomy plus paratracheal lymph node resection, hypertension, smoking, and chronic obstructive pulmonary disease requiring oxygen.
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References
Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105:3768–3785.
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.
Olsen EA, Whittaker S, Kim YH, 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;29:2598–2607.
Lamberg SI, Bunn PA, Jr. Cutaneous T-cell lymphomas. Summary of the Mycosis Fungoides Cooperative Group-National Cancer Institute Workshop. Arch Dermatol. 1979;115:1103–1105.
Fauconneau A, Pham-Ledard A, Cappellen D, et al. Assessment of diagnostic criteria between primary cutaneous anaplastic large-cell lymphoma and CD30-rich transformed mycosis fungoides; a study of 66 cases. Br J Dermatol. 2015;172:1547–1554.
Jawed SI, Myskowski PL, Horwitz S, Moskowitz A, Querfeld C. Primary cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome): part II. Prognosis, management, and future directions. J Am Acad Dermatol. 2014;70:223.e221–217; quiz 240–222.
Horwitz SM, Olsen EA, Duvic M, Porcu P, Kim YH. Review of the treatment of mycosis fungoides and sezary syndrome: a stage-based approach. J Natl Compr Canc Netw. 2008;6:436–442.
Zelenetz AD GL, Wierde WG, et al. NCCN Clinical Practice Guidelines in Oncology: Non- Hodkin's lymphomas. 2015; v 2.2015. https://www.nccn.org/professionals/physician_gls/f_guidelines.asp. Accessed March 7, 2017.
Jawed SI, Myskowski PL, Horwitz S, Moskowitz A, Querfeld C. Primary cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome): part I. Diagnosis: clinical and histopathologic features and new molecular and biologic markers. J Am Acad Dermatol. 2014;70:205.e201–216; quiz 221–202.
Benner MF, Jansen PM, Vermeer MH, Willemze R. Prognostic factors in transformed mycosis fungoides: a retrospective analysis of 100 cases. Blood. 2012;119:1643–1649.
Thomas TO, Agrawal P, Guitart J, et al. Outcome of patients treated with a single-fraction dose of palliative radiation for cutaneous T-cell lymphoma. Int J Radiat Oncol Biol Phys. 2013;85:747–753.
Olsen EA, Rosen ST, Vollmer RT, et al. Interferon alfa-2a in the treatment of cutaneous T cell lymphoma. J Am Acad Dermatol. 1989;20:395–407.
Olsen EA. Interferon in the treatment of cutaneous T-cell lymphoma. Dermatol Ther. 2003;16:311–321.
Kuzel TM, Roenigk HH Jr, Samuelson E, et al. Effectiveness of interferon alfa-2a combined with phototherapy for mycosis fungoides and the Sezary syndrome. J Clin Oncol. 1995;13:257–263.
Stadler R, Otte HG. Combination therapy of cutaneous T cell lymphoma with interferon alpha-2a and photochemotherapy. Recent Results Cancer Res. 1995;139:391–401.
Knobler R, Duvic M, Querfeld C, et al. Long-term follow-up and survival of cutaneous T-cell lymphoma patients treated with extracorporeal photopheresis. Photodermatol Photoimmunol Photomed. 2012;28:250–257.
Duvic M, Hester JP, Lemak NA. Photopheresis therapy for cutaneous T-cell lymphoma. J Am Acad Dermatol. 1996;35:573–579.
Dippel E, Schrag H, Goerdt S, Orfanos CE. Extracorporeal photopheresis and interferon-alpha in advanced cutaneous T-cell lymphoma. Lancet. 1997;350:32–33.
Haley HR, Davis DA, Sams WM. Durable loss of a malignant T-cell clone in a stage IV cutaneous T-cell lymphoma patient treated with high-dose interferon and photopheresis. J Am Acad Dermatol. 1999;41:880–883.
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.
Duvic M, Martin AG, Kim Y, et al. 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;137:581–593.
Querfeld C, Rosen ST, Guitart J, et al. Comparison of selective retinoic acid receptor- and retinoic X receptor-mediated efficacy, tolerance, and survival in cutaneous t-cell lymphoma. J Am Acad Dermatol. 2004;51:25–32.
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;49:873-878.
Zackheim HS, Kashani-Sabet M, Hwang ST. Low-dose methotrexate to treat erythrodermic cutaneous T-cell lymphoma: results in twenty-nine patients. J Am Acad Dermatol. 1996;34:626–631.
McDonald CJ, Bertino JR. Treatment of mycosis fungoides lymphoma: effectiveness of infusions of methotrexate followed by oral citrovorum factor. Cancer Treat Rep. 1978;62:1009–1014.
Wu J, Nihal M, Siddiqui J, Vonderheid EC, Wood GS. Low FAS/CD95 expression by CTCL correlates with reduced sensitivity to apoptosis that can be restored by FAS upregulation. J Invest Dermatol. 2009;129:1165–1173.
Piekarz RL, Frye R, Turner M, 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;27:5410–5417.
Whittaker SJ, Demierre MF, Kim EJ, et al. Final results from a multicenter, international, pivotal study of romidepsin in refractory cutaneous T-cell lymphoma. J Clin Oncol. 2010;28:4485–4491.
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.
Querfeld C, Mehta N, Rosen ST, et al. Alemtuzumab for relapsed and refractory erythrodermic cutaneous T-cell lymphoma: a single institution experience from the Robert H. Lurie Comprehensive Cancer Center. Leuk Lymphoma. 2009;50:1969–1976.
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.
Duvic M, Talpur R, Wen S, Kurzrock R, David CL, Apisarnthanarax N. Phase II evaluation of gemcitabine monotherapy for cutaneous T-cell lymphoma. Clin Lymphoma Myeloma. 2006;7:51–58.
Dummer R, Quaglino P, Becker JC, 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;30:4091–4097.
Duarte RF, Canals C, Onida F, et al. Allogeneic hematopoietic cell transplantation for patients with mycosis fungoides and Sezary syndrome: a retrospective analysis of the Lymphoma Working Party of the European Group for Blood and Marrow Transplantation. J Clin Oncol. 2010;28:4492–4499.
Wu PA, Kim YH, Lavori PW, Hoppe RT, Stockerl-Goldstein KE. A meta-analysis of patients receiving allogeneic or autologous hematopoietic stem cell transplant in mycosis fungoides and Sezary syndrome. Biol Blood Marrow Transplant. 2009;15:982–990.
Kim YH, Tavallaee M, Sundram U, et al. Phase II investigator-initiated study of brentuximab vedotin in mycosis fungoides and Sezary syndrome with variable CD30 expression level: a multi-Institution Collaborative Project. J Clin Oncol. 2015;33:3750–3758.
Horwitz SM, Porcu P, Flinn I, et al. Duvelisib (IPI-145), a phosphoinositide-3-kinase-δ,γ inhibitor, shows activity in patients with relapsed/refractory T-cell lymphoma [ASH abstract 803]. Blood. 2014;124(suppl 21).
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Gonzalez, B.R., Rosen, S.T., Querfeld, C. (2017). Cutaneous T-cell lymphoma. In: Zain, J., Kwak, L. (eds) Management of Lymphomas: A Case-Based Approach. Adis, Cham. https://doi.org/10.1007/978-3-319-26827-9_10
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