Skip to main content
Top
Published in: Current Dermatology Reports 4/2022

05-08-2022 | Lymphoma | Hospital-Based Dermatology (L Guggina and C Nguyen, Section Editors)

Inpatient Considerations in the Diagnosis and Management of the Cutaneous T-Cell Lymphoma Patient

Authors: Samantha Jakuboski, Thomas Doyle, Marianne Tawa, David C. Fisher, Nicole LeBoeuf, Cecilia Larocca

Published in: Current Dermatology Reports | Issue 4/2022

Login to get access

Abstract

Purpose of Review

To review when to consider a diagnosis of cutaneous T-cell lymphoma (CTCL) in the inpatient setting and the diagnostic approach, as well as complications of disease and treatment that could lead to hospitalization.

Recent Findings

Cutaneous T-cell lymphomas (CTCLs) are a diverse group of non-Hodgkin lymphomas that are difficult to diagnosis as the initial presentation can mimic other benign inflammatory or infectious conditions of the skin. While the clinical course is typically indolent, with most cases normally diagnosed and managed in the outpatient settings, some CTCL-associated clinical scenarios, including infections, treatment-related toxicity/side effects, hemophagocytic lymphohistiocytosis (HLH), or spread to extra-cutaneous organs, warrant management in the inpatient setting.

Summary

Early involvement of dermatology specialists is critical for recognizing and diagnosing CTCL in a timely matter, managing treatments and their toxicities, and for improving both morbidity and mortality in patients.
Literature
1.
go back to reference Larocca C, Kupper T. Mycosis fungoides and Sezary syndrome: an update. Hematol Oncol Clin North Am. 2019;33(1):103–20.CrossRef Larocca C, Kupper T. Mycosis fungoides and Sezary syndrome: an update. Hematol Oncol Clin North Am. 2019;33(1):103–20.CrossRef
2.
go back to reference • Kirsch IR, Watanabe R, O'Malley JT, et al. TCR sequencing facilitates diagnosis and identifies mature T cells as the cell of origin in CTCL. Sci Transl Med. 2015;7(308):308ra158. The study demonstrated the success of and introduces high-throughput TCR sequencing as an accurate diagnostic tool for CTCL in all stages and provided insights into the CTCL cell of origin and location. • Kirsch IR, Watanabe R, O'Malley JT, et al. TCR sequencing facilitates diagnosis and identifies mature T cells as the cell of origin in CTCL. Sci Transl Med. 2015;7(308):308ra158. The study demonstrated the success of and introduces high-throughput TCR sequencing as an accurate diagnostic tool for CTCL in all stages and provided insights into the CTCL cell of origin and location.
3.
go back to reference Jackow CM, et al. Association of erythrodermic cutaneous T-cell lymphoma, superantigen-positive Staphylococcus aureus, and oligoclonal T-cell receptor V beta gene expansion. Blood. 1997;89(1):32–40.CrossRef Jackow CM, et al. Association of erythrodermic cutaneous T-cell lymphoma, superantigen-positive Staphylococcus aureus, and oligoclonal T-cell receptor V beta gene expansion. Blood. 1997;89(1):32–40.CrossRef
4.
go back to reference van Doorn R, et al. Mycosis fungoides: disease evolution and prognosis of 309 Dutch patients. Arch Dermatol. 2000;136(4):504–10. van Doorn R, et al. Mycosis fungoides: disease evolution and prognosis of 309 Dutch patients. Arch Dermatol. 2000;136(4):504–10.
5.
go back to reference • Larocca CA, LeBoeuf NR. Overview of cutaneous T-cell lymphomas. Hematol Oncol Clin North Am. 2019;33(4):669–686. Literature review on mycosis fungoides and sézary syndrome. • Larocca CA, LeBoeuf NR. Overview of cutaneous T-cell lymphomas. Hematol Oncol Clin North Am. 2019;33(4):669–686. Literature review on mycosis fungoides and sézary syndrome.
6.
go back to reference Semaan S, et al. A clinician’s guide to cutaneous T-cell lymphoma presenting as recalcitrant eczematous dermatitis in adults. Int J Womens Dermatol. 2021;7(4):422–7.CrossRef Semaan S, et al. A clinician’s guide to cutaneous T-cell lymphoma presenting as recalcitrant eczematous dermatitis in adults. Int J Womens Dermatol. 2021;7(4):422–7.CrossRef
7.
go back to reference Martinez-Escala ME, et al. Progression of undiagnosed cutaneous lymphoma after anti-tumor necrosis factor-alpha therapy. J Am Acad Dermatol. 2018;78(6):1068–76.CrossRef Martinez-Escala ME, et al. Progression of undiagnosed cutaneous lymphoma after anti-tumor necrosis factor-alpha therapy. J Am Acad Dermatol. 2018;78(6):1068–76.CrossRef
8.
go back to reference Marks J. Erythroderma and its management. Clin Exp Dermatol. 1982;7(4):415–22.CrossRef Marks J. Erythroderma and its management. Clin Exp Dermatol. 1982;7(4):415–22.CrossRef
9.
go back to reference Andersen RM, Thyssen JP, Maibach HI. The role of wet wrap therapy in skin disorders - a literature review. Acta Derm Venereol. 2015;95(8):933–9.CrossRef Andersen RM, Thyssen JP, Maibach HI. The role of wet wrap therapy in skin disorders - a literature review. Acta Derm Venereol. 2015;95(8):933–9.CrossRef
10.
go back to reference Pielop JA, Jones D, Duvic M. Transient CD30+ nodal transformation of cutaneous T-cell lymphoma associated with cyclosporine treatment. Int J Dermatol. 2001;40(8):505–11.CrossRef Pielop JA, Jones D, Duvic M. Transient CD30+ nodal transformation of cutaneous T-cell lymphoma associated with cyclosporine treatment. Int J Dermatol. 2001;40(8):505–11.CrossRef
11.
go back to reference Botella-Estrada R, et al. Erythroderma. A clinicopathological study of 56 cases. Arch Dermatol. 1994;130(12):1503–7. Botella-Estrada R, et al. Erythroderma. A clinicopathological study of 56 cases. Arch Dermatol. 1994;130(12):1503–7.
12.
go back to reference Olsen EA, 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.CrossRef Olsen EA, 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.CrossRef
13.
go back to reference Axelrod PI, Lorber B, Vonderheid EC. Infections complicating mycosis fungoides and Sezary syndrome. JAMA. 1992;267(10):1354–8.CrossRef Axelrod PI, Lorber B, Vonderheid EC. Infections complicating mycosis fungoides and Sezary syndrome. JAMA. 1992;267(10):1354–8.CrossRef
14.
go back to reference Tsambiras PE, et al. Infectious complications of cutaneous t-cell lymphoma. Cancer Control. 2001;8(2):185–8.CrossRef Tsambiras PE, et al. Infectious complications of cutaneous t-cell lymphoma. Cancer Control. 2001;8(2):185–8.CrossRef
15.
go back to reference Tokura Y, et al. Cutaneous colonization with staphylococci influences the disease activity of Sezary syndrome: a potential role for bacterial superantigens. Br J Dermatol. 1995;133(1):6–12.CrossRef Tokura Y, et al. Cutaneous colonization with staphylococci influences the disease activity of Sezary syndrome: a potential role for bacterial superantigens. Br J Dermatol. 1995;133(1):6–12.CrossRef
16.
go back to reference Tokura Y, et al. Stimulation of cutaneous T-cell lymphoma cells with superantigenic staphylococcal toxins. J Invest Dermatol. 1992;98(1):33–7.CrossRef Tokura Y, et al. Stimulation of cutaneous T-cell lymphoma cells with superantigenic staphylococcal toxins. J Invest Dermatol. 1992;98(1):33–7.CrossRef
17.
go back to reference Knaak C, et al. Treatment and mortality of hemophagocytic lymphohistiocytosis in adult critically Ill patients: a systematic review with pooled analysis. Crit Care Med. 2020;48(11):e1137–46.CrossRef Knaak C, et al. Treatment and mortality of hemophagocytic lymphohistiocytosis in adult critically Ill patients: a systematic review with pooled analysis. Crit Care Med. 2020;48(11):e1137–46.CrossRef
18.
go back to reference Janka GE, Lehmberg K. Hemophagocytic lymphohistiocytosis: pathogenesis and treatment. Hematology Am Soc Hematol Educ Program. 2013;2013:605–11.CrossRef Janka GE, Lehmberg K. Hemophagocytic lymphohistiocytosis: pathogenesis and treatment. Hematology Am Soc Hematol Educ Program. 2013;2013:605–11.CrossRef
19.
go back to reference Mehta RS, Smith RE. Hemophagocytic lymphohistiocytosis (HLH): a review of literature. Med Oncol. 2013;30(4):740.CrossRef Mehta RS, Smith RE. Hemophagocytic lymphohistiocytosis (HLH): a review of literature. Med Oncol. 2013;30(4):740.CrossRef
20.
go back to reference Filipovich A, McClain K, Grom A. Histiocytic disorders: recent insights into pathophysiology and practical guidelines. Biol Blood Marrow Transplant. 2010;16(1 Suppl):S82–9.CrossRef Filipovich A, McClain K, Grom A. Histiocytic disorders: recent insights into pathophysiology and practical guidelines. Biol Blood Marrow Transplant. 2010;16(1 Suppl):S82–9.CrossRef
21.
go back to reference Fajgenbaum DC, June CH. Cytokine storm. N Engl J Med. 2020;383(23):2255–73.CrossRef Fajgenbaum DC, June CH. Cytokine storm. N Engl J Med. 2020;383(23):2255–73.CrossRef
22.
go back to reference Lee DE, et al. Hemophagocytic lymphohistiocytosis in cutaneous T-cell lymphoma. JAMA Dermatol. 2018;154(7):828–31.CrossRef Lee DE, et al. Hemophagocytic lymphohistiocytosis in cutaneous T-cell lymphoma. JAMA Dermatol. 2018;154(7):828–31.CrossRef
23.
go back to reference Fardet L, et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol. 2014;66(9):2613–20.CrossRef Fardet L, et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol. 2014;66(9):2613–20.CrossRef
24.
go back to reference Henter JI, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48(2):124–31.CrossRef Henter JI, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48(2):124–31.CrossRef
25.
go back to reference Jordan MB, et al. How I treat hemophagocytic lymphohistiocytosis. Blood. 2011;118(15):4041–52.CrossRef Jordan MB, et al. How I treat hemophagocytic lymphohistiocytosis. Blood. 2011;118(15):4041–52.CrossRef
26.
go back to reference Bergsten E, et al. Confirmed efficacy of etoposide and dexamethasone in HLH treatment: long-term results of the cooperative HLH-2004 study. Blood. 2017;130(25):2728–38.CrossRef Bergsten E, et al. Confirmed efficacy of etoposide and dexamethasone in HLH treatment: long-term results of the cooperative HLH-2004 study. Blood. 2017;130(25):2728–38.CrossRef
27.
go back to reference Marsh RA, et al. Salvage therapy of refractory hemophagocytic lymphohistiocytosis with alemtuzumab. Pediatr Blood Cancer. 2013;60(1):101–9.CrossRef Marsh RA, et al. Salvage therapy of refractory hemophagocytic lymphohistiocytosis with alemtuzumab. Pediatr Blood Cancer. 2013;60(1):101–9.CrossRef
28.
go back to reference Maschalidi S, et al. Therapeutic effect of JAK1/2 blockade on the manifestations of hemophagocytic lymphohistiocytosis in mice. Blood. 2016;128(1):60–71.CrossRef Maschalidi S, et al. Therapeutic effect of JAK1/2 blockade on the manifestations of hemophagocytic lymphohistiocytosis in mice. Blood. 2016;128(1):60–71.CrossRef
29.
go back to reference Henter JI, et al. Hypercytokinemia in familial hemophagocytic lymphohistiocytosis. Blood. 1991;78(11):2918–22.CrossRef Henter JI, et al. Hypercytokinemia in familial hemophagocytic lymphohistiocytosis. Blood. 1991;78(11):2918–22.CrossRef
30.
go back to reference Yu JT, et al. Reduced intensity conditioning allogeneic hematopoietic stem cell transplant could be beneficial to angioimmunoblastic T-cell lymphoma patients with hemophagocytic lymphohistiocytosis. Ann Hematol. 2012;91(5):805–7.CrossRef Yu JT, et al. Reduced intensity conditioning allogeneic hematopoietic stem cell transplant could be beneficial to angioimmunoblastic T-cell lymphoma patients with hemophagocytic lymphohistiocytosis. Ann Hematol. 2012;91(5):805–7.CrossRef
31.
go back to reference Baser S, et al. Pulmonary manifestations in patients with cutaneous T-cell lymphomas. Cancer. 2007;109(8):1550–5.CrossRef Baser S, et al. Pulmonary manifestations in patients with cutaneous T-cell lymphomas. Cancer. 2007;109(8):1550–5.CrossRef
32.
go back to reference Stein M, et al. Central neurologic involvement in mycosis fungoides: ten cases, actuarial risk assessment, and predictive factors. Cancer J. 2006;12(1):55–62.CrossRef Stein M, et al. Central neurologic involvement in mycosis fungoides: ten cases, actuarial risk assessment, and predictive factors. Cancer J. 2006;12(1):55–62.CrossRef
33.
go back to reference Zhao G, et al. Central nervous system involvement in cutaneous T-cell lymphoma: 2 illustrative cases and a review of current literature. Clin Lymphoma Myeloma Leuk. 2014;14(1):e25-30.CrossRef Zhao G, et al. Central nervous system involvement in cutaneous T-cell lymphoma: 2 illustrative cases and a review of current literature. Clin Lymphoma Myeloma Leuk. 2014;14(1):e25-30.CrossRef
34.
go back to reference Vu BA, Duvic M. Central nervous system involvement in patients with mycosis fungoides and cutaneous large-cell transformation. J Am Acad Dermatol. 2008;59(2 Suppl 1):S16-22.CrossRef Vu BA, Duvic M. Central nervous system involvement in patients with mycosis fungoides and cutaneous large-cell transformation. J Am Acad Dermatol. 2008;59(2 Suppl 1):S16-22.CrossRef
35.
go back to reference Duarte RF, et al. Long-term outcome of allogeneic hematopoietic cell transplantation for patients with mycosis fungoides and Sezary syndrome: a European society for blood and marrow transplantation lymphoma working party extended analysis. J Clin Oncol. 2014;32(29):3347–8.CrossRef Duarte RF, et al. Long-term outcome of allogeneic hematopoietic cell transplantation for patients with mycosis fungoides and Sezary syndrome: a European society for blood and marrow transplantation lymphoma working party extended analysis. J Clin Oncol. 2014;32(29):3347–8.CrossRef
36.
go back to reference Foss FM. DAB(389)IL-2 (ONTAK): a novel fusion toxin therapy for lymphoma. Clin Lymphoma 2000;1(2):110–6, discussion 117. Foss FM. DAB(389)IL-2 (ONTAK): a novel fusion toxin therapy for lymphoma. Clin Lymphoma 2000;1(2):110–6, discussion 117.
37.
go back to reference Ohmachi K, et al. E7777 in Japanese patients with relapsed/refractory peripheral and cutaneous T-cell lymphoma: a phase I study. Cancer Sci. 2018;109(3):794–802.CrossRef Ohmachi K, et al. E7777 in Japanese patients with relapsed/refractory peripheral and cutaneous T-cell lymphoma: a phase I study. Cancer Sci. 2018;109(3):794–802.CrossRef
38.
go back to reference Kawai H, et al. Phase II study of E7777 in Japanese patients with relapsed/refractory peripheral and cutaneous T-cell lymphoma. Cancer Sci. 2021;112(6):2426–35.CrossRef Kawai H, et al. Phase II study of E7777 in Japanese patients with relapsed/refractory peripheral and cutaneous T-cell lymphoma. Cancer Sci. 2021;112(6):2426–35.CrossRef
39.
go back to reference Duvic M, et al. Phase 1/2 study of mogamulizumab, a defucosylated anti-CCR4 antibody, in previously treated patients with cutaneous T-cell lymphoma. Blood. 2015;125(12):1883–9.CrossRef Duvic M, et al. Phase 1/2 study of mogamulizumab, a defucosylated anti-CCR4 antibody, in previously treated patients with cutaneous T-cell lymphoma. Blood. 2015;125(12):1883–9.CrossRef
40.
go back to reference Kim YH, et al. Mogamulizumab versus vorinostat in previously treated cutaneous T-cell lymphoma (MAVORIC): an international, open-label, randomised, controlled phase 3 trial. Lancet Oncol. 2018;19(9):1192–204.CrossRef Kim YH, et al. Mogamulizumab versus vorinostat in previously treated cutaneous T-cell lymphoma (MAVORIC): an international, open-label, randomised, controlled phase 3 trial. Lancet Oncol. 2018;19(9):1192–204.CrossRef
41.
go back to reference Larocca C, Kupper TS, LeBoeuf NR. Mogamulizumab forecast: clearer patients, with a slight chance of immune mayhem. Clin Cancer Res. 2019;25(24):7272–4.CrossRef Larocca C, Kupper TS, LeBoeuf NR. Mogamulizumab forecast: clearer patients, with a slight chance of immune mayhem. Clin Cancer Res. 2019;25(24):7272–4.CrossRef
42.
go back to reference Chen L, et al. Mogamulizumab-associated cutaneous granulomatous drug eruption mimicking mycosis fungoides but possibly indicating durable clinical response. JAMA Dermatol. 2019;155(8):968–71.CrossRef Chen L, et al. Mogamulizumab-associated cutaneous granulomatous drug eruption mimicking mycosis fungoides but possibly indicating durable clinical response. JAMA Dermatol. 2019;155(8):968–71.CrossRef
43.
go back to reference Kwan JM, et al. Mogamulizumab-associated acute myocarditis in a patient with T-cell lymphoma. JACC Case Rep. 2021;3(7):1018–23.CrossRef Kwan JM, et al. Mogamulizumab-associated acute myocarditis in a patient with T-cell lymphoma. JACC Case Rep. 2021;3(7):1018–23.CrossRef
44.
go back to reference Larocca CA, TM, LeBoeuf N, Virgin C, Goyal A, Amato A, De Girolami U, McGrath CKE, Mullally W, Sparks J, Fisher DC, Kupper TS. Successful treatment of mogamulizumab-induced myositis. USCLC annual meeting. 2021. Larocca CA, TM, LeBoeuf N, Virgin C, Goyal A, Amato A, De Girolami U, McGrath CKE, Mullally W, Sparks J, Fisher DC, Kupper TS. Successful treatment of mogamulizumab-induced myositis. USCLC annual meeting. 2021.
45.
go back to reference Roccuzzo G, et al. Immune check point inhibitors in primary cutaneous T-cell lymphomas: biologic rationale, clinical results and future perspectives. Front Oncol. 2021;11: 733770.CrossRef Roccuzzo G, et al. Immune check point inhibitors in primary cutaneous T-cell lymphomas: biologic rationale, clinical results and future perspectives. Front Oncol. 2021;11: 733770.CrossRef
46.
go back to reference Lesokhin AM, et al. Nivolumab in patients with relapsed or refractory hematologic malignancy: preliminary results of a phase Ib study. J Clin Oncol. 2016;34(23):2698–704.CrossRef Lesokhin AM, et al. Nivolumab in patients with relapsed or refractory hematologic malignancy: preliminary results of a phase Ib study. J Clin Oncol. 2016;34(23):2698–704.CrossRef
47.
go back to reference Khodadoust MS, et al. Pembrolizumab in relapsed and refractory mycosis fungoides and Sezary syndrome: a multicenter phase II study. J Clin Oncol. 2020;38(1):20–8.CrossRef Khodadoust MS, et al. Pembrolizumab in relapsed and refractory mycosis fungoides and Sezary syndrome: a multicenter phase II study. J Clin Oncol. 2020;38(1):20–8.CrossRef
48.
go back to reference •• Weng WK, Arai S, Rezvani A, et al. Nonmyeloablative allogeneic transplantation achieves clinical and molecular remission in cutaneous T-cell lymphoma. Blood Adv. 2020;4(18):4474–4482. Pivotal clinical trial that showed allogeneic stem cell transplant to be an effective treatment in cutaneous T-cell lymphoma. •• Weng WK, Arai S, Rezvani A, et al. Nonmyeloablative allogeneic transplantation achieves clinical and molecular remission in cutaneous T-cell lymphoma. Blood Adv. 2020;4(18):4474–4482. Pivotal clinical trial that showed allogeneic stem cell transplant to be an effective treatment in cutaneous T-cell lymphoma.
49.
go back to reference Divito SJ, et al. Peripheral host T cells survive hematopoietic stem cell transplantation and promote graft-versus-host disease. J Clin Invest. 2020;130(9):4624–36.CrossRef Divito SJ, et al. Peripheral host T cells survive hematopoietic stem cell transplantation and promote graft-versus-host disease. J Clin Invest. 2020;130(9):4624–36.CrossRef
50.
go back to reference Foo SH, et al. Unmasking mycosis fungoides/Sezary syndrome from preceding or co-existing benign inflammatory dermatoses requiring systemic therapies: patients frequently present with advanced disease and have an aggressive clinical course. Br J Dermatol. 2016;174(4):901–4.CrossRef Foo SH, et al. Unmasking mycosis fungoides/Sezary syndrome from preceding or co-existing benign inflammatory dermatoses requiring systemic therapies: patients frequently present with advanced disease and have an aggressive clinical course. Br J Dermatol. 2016;174(4):901–4.CrossRef
51.
go back to reference Weenig RH, et al. Fatal cytotoxic cutaneous lymphoma presenting as ulcerative psoriasis. Arch Dermatol. 2009;145(7):801–8.CrossRef Weenig RH, et al. Fatal cytotoxic cutaneous lymphoma presenting as ulcerative psoriasis. Arch Dermatol. 2009;145(7):801–8.CrossRef
Metadata
Title
Inpatient Considerations in the Diagnosis and Management of the Cutaneous T-Cell Lymphoma Patient
Authors
Samantha Jakuboski
Thomas Doyle
Marianne Tawa
David C. Fisher
Nicole LeBoeuf
Cecilia Larocca
Publication date
05-08-2022
Publisher
Springer US
Published in
Current Dermatology Reports / Issue 4/2022
Electronic ISSN: 2162-4933
DOI
https://doi.org/10.1007/s13671-022-00367-4

Other articles of this Issue 4/2022

Current Dermatology Reports 4/2022 Go to the issue

Covid-19 in Dermatology (J. M. Gelfand, Section editor)

Outpatient Management of COVID-19: A Primer for the Dermatologist

Covid-19 in Dermatology (J. M. Gelfand, Section editor)

Impact of the COVID-19 Pandemic on the Delivery of Dermatological Care

Contact Dermatitis (B.Adler and V. Deleo, Section editors)

Contact Urticaria Syndrome: a Comprehensive Review

Contact Dermatitis (Brandon Adler and Vincent Deleo, Section editors)

Contact Dermatitis in the Inpatient Hospital Setting–an Updated Review of the Literature