Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part II: Prognosis and management - 12/10/21

Doi : 10.1016/j.jaad.2021.04.081

Yuna Oh, BS, Joseph R. Stoll, BTL, Alison Moskowitz, MD, Melissa Pulitzer, MD, Steven Horwitz, MD, Patricia Myskowski, MD, Sarah J. Noor, MD ^⁎
Memorial Sloan Kettering Cancer Center, New York, New York

^∗Correspondence to: Sarah J. Noor, MD, 530 E 74th Street, New York, NY 10021.530 E 74th StreetNew YorkNY10021

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.

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Key words : adult T-cell leukemia, adult T-cell lymphoma, angioimmunoblastic T-cell lymphoma, brentuximab, CD30⁺ lymphoproliferative disorders, cutaneous T-cell lymphoma, cytotoxic T-cell lymphoma, extranodal NK/T-cell lymphoma, immunomodulators, mycosis fungoides, nasal type, non-MF/SS, peripheral T-cell lymphoma, not otherwise specified, phototherapy, 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, prognosis, Sézary syndrome, skin-directed treatment, staging, subcutaneous panniculitis-like T-cell lymphoma, systemic treatment, targeted therapies, topical corticosteroids

Abbreviations used : AE, ATCL, ATLL, BV, CD30⁺ LPD, CI, CR, CT, CTCL, EBV, ENKTL, HPS, HSCT, LyP, MF, MTX, ORR, OS, PC-ALCL, PCSM-TCLPD, PET, RT, SPTCL, SS

Plan

Introduction

General overview: evaluation and staging

Primary cutaneous CD30⁺ lymphoproliferative disorders

Lymphomatoid papulosis

Primary cutaneous anaplastic large-cell lymphoma

Primary cutaneous CD4⁺ small-medium sized T-cell lymphoproliferative disorder/Primary cutaneous acral CD8⁺ T-cell lymphoma

Prognosis

Management

Subcutaneous panniculitis-like T-cell lymphoma

Prognosis

Management

Cytotoxic T-cell lymphomas

Primary cutaneous gamma-delta T-cell lymphoma

Primary cutaneous CD8⁺ aggressive epidermotropic cytotoxic T-cell lymphoma

Other T-cell lymphomas presenting in the skin—Prognosis and management

Peripheral T-cell lymphoma-not otherwise specified/angioimmunoblastic T-cell lymphoma

Extranodal NK/T-cell lymphoma-nasal type and chronic active EBV infection

ATLL

Conclusion

	Funding sources: None.
	IRB approval status: Not applicable.
	Date of release: November 2021.
	Expiration date: November 2024.
	Reprints not available from the authors.