Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part II: Prognosis and management Review
| Authors: | Oh, Y.; Stoll, J. R.; Moskowitz, A.; Pulitzer, M.; Horwitz, S.; Myskowski, P.; Noor, S. J. |
| Review Title: | Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part II: Prognosis and management |
| Abstract: | Primary cutaneous T-cell lymphomas (CTCLs) other than mycosis fungoides (MF) and Sézary syndrome (SS) encompass a heterogenous group of non-Hodgkin lymphomas with variable clinical courses, prognoses, and management approaches. Given the morphologic and histologic overlap among the CTCL subtypes and other T-cell lymphomas with cutaneous manifestations, thorough evaluation with clinicopathologic correlation and exclusion of systemic involvement are essential prior to initiating therapy. Staging and treatment recommendations vary, depending on the subtype, clinical behavior, and treatment response. Generally, for subtypes in which staging is recommended, Ann Arbor or tumor, node, metastasis staging specific to CTCL other than MF or SS are used. For many subtypes, there is no standard treatment to date. Available recommended treatments range widely, from no active or minimal intervention with skin-directed therapy to aggressive systemic therapies that include multi-agent chemotherapy with consideration for hematopoietic stem cell transplant. Emerging targeted therapies, such as brentuximab, a chimeric antibody targeting CD30, show promise in altering the disease course of non-MF/SS CTCLs. © 2021 American Academy of Dermatology, Inc. |
| Keywords: | staging; cutaneous t-cell lymphoma; mycosis fungoides; targeted therapies; immunomodulators; peripheral t-cell lymphoma; subcutaneous panniculitis-like t-cell lymphoma; phototherapy; angioimmunoblastic t-cell lymphoma; not otherwise specified; nasal type; prognosis; skin-directed treatment; systemic treatment; topical corticosteroids; brentuximab; extranodal nk/t-cell lymphoma; adult t-cell leukemia; sézary syndrome; adult t-cell lymphoma; cd30+ lymphoproliferative disorders; cytotoxic t-cell lymphoma; non-mf/ss; primary cutaneous acral cd8+ t-cell lymphoma; primary cutaneous cd4+ small/medium t-cell lymphoproliferative disorder; primary cutaneous cd8+ aggressive epidermotropic cytotoxic t-cell lymphoma; primary cutaneous gamma-delta t-cell lymphoma |
| Journal Title: | Journal of the American Academy of Dermatology |
| Volume: | 85 |
| Issue: | 5 |
| ISSN: | 0190-9622 |
| Publisher: | Mosby Elsevier |
| Date Published: | 2021-11-01 |
| Start Page: | 1093 |
| End Page: | 1106 |
| Language: | English |
| DOI: | 10.1016/j.jaad.2021.04.081 |
| PUBMED: | 33945836 |
| PROVIDER: | scopus |
| DOI/URL: | |
| Notes: | Review -- For Part I, see https://synapse.mskcc.org/synapse/works/190804 -- Source: Scopus |
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