Recurrent Fevers and Painful Subcutaneous Nodules: A Case of Subcutaneous Panniculits-like T-Cell Lymphoma

Rachel L. Axman¹, Brian Wolf², Susan Lin², Benjamin Schwartz², Urmi Khanna², Fahad Zafar², Melvyn Hecht² and Isabel M. McFarlane^1,

¹Department of Internal Medicine, State University of New York, Downstate Health Sciences University, Brooklyn, NY, USA 11203

²Department of Medicine, Maimonides Medical Center, Department of Medicine, Brooklyn, NY 11219

Cite this paper

Rachel L. Axman, Brian Wolf, Susan Lin, Benjamin Schwartz, Urmi Khanna, Fahad Zafar, Melvyn Hecht and Isabel M. McFarlane. Recurrent Fevers and Painful Subcutaneous Nodules: A Case of Subcutaneous Panniculits-like T-Cell Lymphoma. American Journal of Medical Case Reports. 2021; 9(7):367-371. doi: 10.12691/AJMCR-9-7-7

Abstract

Background: Subcutaneous Panniculitis-Like T-Cell Lymphoma is a cytotoxic T-cell lymphoma that infiltrates subcutaneous adipose tissue and rarely involves the lymph nodes. SPTCL accounts for less than 1% of non-Hodgkin’s lymphomas. Lesions are commonly localized to the trunk and lower extremities and may be relapsing and remitting in nature. Systemic symptoms such as fever and night sweats are common. SPTCL may closely mimic cellulitis, and other causes of skin and soft tissue infections. Definitive diagnosis is made with tissue biopsy showing atypical lymphocytes rimming adipocytes. There is no current standard of treatment. Prognosis is favorable with a 5-year survival rate as high as 91%. Case Report: A 40-year-old Chinese female presented with a two-week history of recurrent fevers and painful subcutaneous nodules and plaques, initially thought to be infectious in nature. Tissue sampling demonstrated CD4+ and CD8+ T-cells rimming adipocytes with a high Ki-67 proliferation index and an alpha beta T-cell receptor subtype. A diagnosis of SPTCL was established and the patient was treated with multiagent chemotherapy regimen consisting of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP therapy), which resulted in resolution of her constitutional symptoms and cutaneous. Conclusion: SPTCL is a challenging diagnosis due to the wide array of etiologies of panniculitides and its close clinical resemblance to soft tissue infection. Early tissue biopsy should be employed to expedite management.

Keywords

subcutaneous panniculitis-like t-cell lymphoma, subcutaneous nodules, CD4+/CD8+ T-cells rimming adipocytes

Copyright

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References

[1]	Gonzalez CL, Medeiros LJ, Braziel RM, et al. T-cell lymphoma involving subcutaneous tissue: a clinicopathologic entity commonly associated with hemophagocytic syndrome. Am J Surg Pathol. 1991; 15: 17-27.
[2]	Parveen Z, Thompson K. Subcutaneous Panniculitis-like T-Cell Lymphoma: Redefinition of Diagnostic Criteria in the Recent World Health Organization–European Organization for Research and Treatment of Cancer Classification for Cutaneous Lymphomas. Arch Pathol Lab Med. 1 February 2009; 133(2): 303-308.
[3]	Willemze R, Jansen PM, Cerroni L, et al. Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 Cases. Blood. 2008 Jan 15; 111(2): 838-45.
[4]	Dobos G, Pohrt A, Ram-Wolff C, et al. Epidemiology of Cutaneous T-Cell Lymphomas: A Systematic Review and Meta-Analysis of 16,953 Patients. Cancers (Basel). 2020 Oct 11; 12(10): 2921.
[5]	Musick SR, Lynch DT. Subcutaneous Panniculitis Like T-cell Lymphoma. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan. 2020 Nov 9.
[6]	López-Lerma I, Peñate Y, Gallardo F, et al. Subcutaneous panniculitis-like T-cell lymphoma: Clinical features, therapeutic approach, and outcome in a case series of 16 patients. J Am Acad Dermatol. 2018 Nov; 79(5): 892-898.
[7]	Jaffe ES. The 2008 WHO classification of lymphomas: implications for clinical practice and translational research. Hematology Am Soc Hematol Educ Program. 2009; 523-31.
[8]	Requena L, Yus ES. Panniculitis. Part II. Mostly lobular panniculitis. J Am Acad Dermatol. 2001 Sep; 45(3): 325-61; quiz 362-4.
[9]	Jaffe ES, Harris NL, Stein H, Vardiman JW. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues. World Health Organization Classification of Tumours. Lyon, France: IARC Press, 2001; pp. 351–352
[10]	Gonzalez EG, Selvi E, Lorenzini S, et al. Subcutaneous panniculitis-like T-cell lymphoma misdiagnosed as lupus erythematosus panniculitis. Clin Rheumatol. 2007; 26: 244-246.
[11]	Guenova E, Schanz S, Hoetzenecker W, et al. Systemic corticosteroids for subcutaneous panniculitis-like T-cell lymphoma. Br J Dermatol. 2014 Oct; 171(4): 891-4.
[12]	Hashimoto R, Uchiyama M, Maeno T. Case report of subcutaneous panniculitis-like T-cell lymphoma complicated by eyelid swelling. BMC Ophthalmol. 2016; 16: 117. Published 2016 Jul 20.
[13]	Rojnuckarin P, Nakorn TN, Assanasen T, et al. Cyclosporin in subcutaneous panniculitis-like T-cell lymphoma. Leuk Lymphoma. 2007 Mar; 48(3): 560-3.
[14]	Gochhait, D., Kekade, S., Devi, D., Srinivas, B. H., Siddaraju, N., & Chandrashekar, L. (2019). Subcutaneous Panniculitis-Like T Cell Lymphoma: Approach to Differential Diagnosis on Cytology. Journal of Adolescent and Young Adult Oncology.
[15]	Fujii K. New Therapies and Immunological Findings in Cutaneous T-Cell Lymphoma. Front Oncol. 2018 Jun; 8: 198.
[16]	Mizutani S, Kuroda J, Shimura Y, et al. Cyclosporine A for chemotherapy-resistant subcutaneous panniculitis-like T cell lymphoma with hemophagocytic syndrome. Acta Haematol. 2011; 126(1): 8-12.
[17]	Mehta N, Wayne AS, Kim YH, et al. Bexarotene is active against subcutaneous panniculitis-like T-cell lymphoma in adult and pediatric populations. Clin Lymphoma Myeloma Leuk. 2012 Feb; 12(1): 20-5.
[18]	Michonneau D, Petrella T, Ortonne N, et al. Subcutaneous Panniculitis-like T-cell Lymphoma: Immunosuppressive Drugs Induce Better Response than Polychemotherapy. Acta Derm Venereol. 2017; 97(3): 358-364.
[19]	Zhang H, Gupta R, Wang JC, et al. Subcutaneous panniculitis-like T-cell lymphoma in a patient with long-term remission with standard chemotherapy. J Natl Med Assoc. 2007 Oct; 99(10): 1190-2.