Update on primary cutaneous T-cell lymphomas rare subtypes


Published: 7 May 2024
Abstract Views: 138
PDF: 155
Publisher's note
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.

Authors

Rare subtypes of cutaneous T-cell lymphomas (CTCL) include four entities, primary cutaneous γδ T-cell lymphoma, primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma, and primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorders, primary cutaneous acral CD8+ T-cell lymphoma, which were previously considered provisional and are now included in the new 5th World Health Organization classification of hematolymphoid tumors as distinct entities. An updated summary of the clinical, histological, and genomic characteristics of these uncommon CTCL subtypes is given in this review, with a focus on the growing body of knowledge regarding their classification and possible treatment strategies.


Elder DE, Massi D, Scolyer RA, Willemze R. WHO classification of skin tumors. Lyon, France: IARC Press; 2018.

Swerdlow SH, Campo E, Harris NL, et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. 4th Edition. Lyon, France: IARC Press; 2008.

Swerdlow SH, Campo E, Harris NL, et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Revised 4th edition. Lyon: World Health Organization; 2017:585.

Willemze R, Cerroni L, Kempf W, et al. The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas. Blood 2019;133:1703-14. DOI: https://doi.org/10.1182/blood-2018-11-881268

Alaggio R, Amador C, Anagnostopoulos I, et al. The 5th edition of the World Health Organization classification of haematolymphoid tumours: lymphoid neoplasms. Leukemia 2022;36:1720-48. DOI: https://doi.org/10.1038/s41375-022-01620-2

Berti E, Cerri A, Cavicchini S, et al. Primary cutaneous gamma/delta T-cell lymphoma presenting as disseminated pagetoid reticulosis. J Invest Dermatol 1991;96:718-23. DOI: https://doi.org/10.1111/1523-1747.ep12470953

Burg G, Dummer R, Wilhelm M, et al. A subcutaneous deltapositive T-cell lymphoma that produces interferon gamma. N Engl J Med 1991;325:1078-81. DOI: https://doi.org/10.1056/NEJM199110103251506

Torres-Cabala CA, Huen A, Iyer SP, Miranda RN. Gamma/delta phenotype in primary cutaneous T-cell lymphomas and lymphoid proliferations: challenges for diagnosis and classification. Surg Pathol Clin 2021;14:177-94. DOI: https://doi.org/10.1016/j.path.2021.03.001

Alberti-Violetti S, Maronese CA, Venegoni L, et al. Primary cutaneous gamma-delta T cell lymphomas: a case series and overview of the literature. Dermatopathology 2021;8:515-24. DOI: https://doi.org/10.3390/dermatopathology8040054

Merrill ED, Tetzla???? MT, Young KH, et al. Primary cutaneous T-Cell lymphomas showing gamma-delta (γδ) phenotype and predominantly epidermotropic pattern are clinicopathological distinct from classic primary cutaneous γδ T-cell lymphomas. Am J Surg Pathol 2017;41:204-15. DOI: https://doi.org/10.1097/PAS.0000000000000768

Toro JR, Liewehr DJ, Pabby N, et al. Gamma-delta T-cell phenotype is associated with significantly decreased survival in cutaneous T-cell lymphoma. Blood 2003;101:3407-12. DOI: https://doi.org/10.1182/blood-2002-05-1597

Daniels J, Doukas PG, Escala MEM, et al. Cellular origins and genetic landscape of cutaneous gamma delta T cell lymphomas. Nat Commun 2020;11:1806. DOI: https://doi.org/10.1038/s41467-020-15572-7

Guitart J, Weisenburger DD, Wood G, et al. Cutaneous gd Tcell Lymphomas. Am J Surg Pathol 2012;36:10. DOI: https://doi.org/10.1097/PAS.0b013e31826a5038

Küçük C, Jiang B, Hu X, et al. Activating mutations of STAT5B and STAT3 in lymphomas derived from γδ-T or NK cells. Nat Commun 2015;6:6025. DOI: https://doi.org/10.1038/ncomms7025

Goyal A, O’Leary D, Duncan LM. The significance of epidermal involvement in primary cutaneous gamma/delta (γδ) Tcell lymphoma: A systematic review and meta-analysis. J Cutan Pathol 2021;48:1449-54. DOI: https://doi.org/10.1111/cup.14082

Onkarappa Mangala Y, Onukogu ID, Breen CM, Colvin GA. Primary Cutaneous Gamma-Delta T-cell Lymphoma: A Case Report and Review of Literature. Cureus 2023;15:e35442. DOI: https://doi.org/10.7759/cureus.35442

Muhsen IN, El Fakih R, Hamadani M, et al. Clinical, Diagnostic and prognostic characteristics of primary cutaneous gamma delta t-cell lymphomas. Clin Hematol Int 2022;4:1-10. DOI: https://doi.org/10.1007/s44228-022-00011-9

Voruz S, de Leval L, Cairoli A. Successful salvage therapy for refractory primary cutaneous gamma-delta T-cell lymphoma with a combination of brentuximab vedotin and gemcitabine. Exp Hematol Oncol 2021;10:32. DOI: https://doi.org/10.1186/s40164-021-00225-2

Kinberger M, Ghoreschi F, Moritz R, et al. Primary cutaneous gamma-delta lymphoma responding only to mogamulizumab and total skin electron beam therapy. Eur J Cancer 2022;173:S44. DOI: https://doi.org/10.1016/S0959-8049(22)00640-2

Berti E, Tomasini D, Vermeer MH, et al. Primary cutaneous CD8-positive epidermotropic cytotoxic T cell lymphomas. Am J Pathol 1999;155:483-92. DOI: https://doi.org/10.1016/S0002-9440(10)65144-9

Wei R, Wang H, Zhang J, et al. Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma in a 4- year-old girl. Int J Dermatol 2024. Available from: https://doi.org/10.1111/ijd.16985 DOI: https://doi.org/10.1111/ijd.16985

Flora A, Gibson M, Toon C, et al. Primary cutaneous CD8+ aggressive epidermotropic T-cell lymphoma mimicking pyoderma gangrenosum. Indian J Dermatol Venereol Leprol 2021;88:90-2. DOI: https://doi.org/10.25259/IJDVL_296_2021

Tomasini C, Novelli M, Fanoni D, Berti EF. Erythema multiforme-like lesions in primary cutaneous aggressive cytotoxic epidermotropic CD8+ T-cell lymphoma: A diagnostic and therapeutic challenge. J Cutan Pathol 2017;44:867-73. DOI: https://doi.org/10.1111/cup.12995

Gilkey TW, Pettit C, Korman A, et al. Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma resembling pemphigus vulgaris. Int J Dermatol 2022;61:1298-300. DOI: https://doi.org/10.1111/ijd.15934

Weenig RH, Comfere NI, Gibson LE, et al. Fatal cytotoxic cutaneous lymphoma presenting as ulcerative psoriasis. Arch Dermatol 2009;145:801-8. DOI: https://doi.org/10.1001/archdermatol.2009.119

Guitart J, Martinez-Escala ME, Subtil A, et al. Primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphomas: reappraisal of a provisional entity in the 2016 WHO classification of cutaneous lymphomas. Modern Pathol 2017;30:761-72. DOI: https://doi.org/10.1038/modpathol.2016.240

Fanoni D, Corti L, Alberti-Violetti S, et al. Array-based CGH of primary cutaneous CD8+ aggressive EPIDERMO-tropic cytotoxic T-cell lymphoma. Genes Chromosomes Cancer 2018;57:622-9. DOI: https://doi.org/10.1002/gcc.22673

Torres ANB, Cats D, Out-Luiting JJ, et al. Deregulation of JAK2 signaling underlies primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma. Haematologica 2022;107:702-14. DOI: https://doi.org/10.3324/haematol.2020.274506

Friedmann D, Wechsler J, Delfau MH, et al. Primary cutaneous pleomorphic small T-cell lymphoma: a review of 11 cases. Arch Dermatol 1995;131:1009-15. DOI: https://doi.org/10.1001/archderm.131.9.1009

Alberti-Violetti S, Torres-Cabala CA, Talpur R, et al. Clinicopathological and molecular study of primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma. J Cutan Pathol 2016;43:1121-30. DOI: https://doi.org/10.1111/cup.12806

Rodríguez Pinilla SM, Roncador G, Rodríguez-Peralto JL, et al. Primary Cutaneous CD4+ Small/Medium-sized Pleomorphic T-cell Lymphoma Expresses Follicular T-cell Markers. Am J Surg Pathol 2009;33:81. DOI: https://doi.org/10.1097/PAS.0b013e31818e52fe

Wang L, Rocas D, Dalle S, et al. Primary cutaneous peripheral T-cell lymphomas with a T-follicular helper phenotype: an integrative clinical, pathological and molecular case series study*. Br J Dermatol 2022;187:970-80. DOI: https://doi.org/10.1111/bjd.21791

Spigariolo CB, Berti E, Cerri A, et al. T follicular helper phenotype mycosis fungoides associated with acanthosis nigricans. J Cutan Pathol 2023;50:420-4. DOI: https://doi.org/10.1111/cup.14405

Soderquist CR, Hsiao S, Pang J, et al. Whole exome sequencing of primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorders. Hematol Oncol 2023;41:768-70. DOI: https://doi.org/10.1002/hon.3133

Petrella T, Maubec E, Cornillet-Lefebvre P, et al. Indolent CD8-positive lymphoid proliferation of the ear: a distinct primary cutaneous T-cell lymphoma? Am J Surg Pathol 2007;31:1887-92. DOI: https://doi.org/10.1097/PAS.0b013e318068b527

Stephan C, Grossman ME, Magro CM. Primary cutaneous acral CD8-positive T-cell lymphoproliferative disorder: A clinical and histologic retrospective cohort study. Clinics in dermatology. Clin Dermatol 2023;41:666-79. DOI: https://doi.org/10.1016/j.clindermatol.2023.09.002

Alberti-Violetti S, Fanoni D, Provasi M, et al. Primary cutaneous acral CD8 positive T-cell lymphoma with extra-cutaneous involvement: a long-standing case with an unexpected progression. J Cutan Pathol 2017;44:964-8. DOI: https://doi.org/10.1111/cup.13020

Maubec E, Marinho E, Laroche L, et al. Primary cutaneous acral CD8+ T-cell lymphomas relapse more frequently in younger patients. Br J Haematol 2019;185:598-601. DOI: https://doi.org/10.1111/bjh.15572

Alberti-Violetti, S., & Berti, E. (2024). Update on primary cutaneous T-cell lymphomas rare subtypes. Dermatology Reports, 16(s2). https://doi.org/10.4081/dr.2024.9961

Downloads

Download data is not yet available.

Citations