Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part I: Clinical and histologic features and diagnosis - 12/10/21

Doi : 10.1016/j.jaad.2021.04.080

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

^∗Reprint requests: Sarah J. Noor, MD, Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, 530 E 74th St, New York, NY 10021.Dermatology ServiceDepartment of MedicineMemorial Sloan Kettering Cancer Center530 E 74th StNew YorkNY10021

Abstract

Primary cutaneous T-cell lymphomas (CTCLs) are defined as lymphomas with a T-cell phenotype that present in the skin without evidence of systemic or extracutaneous disease at initial presentation. CTCLs other than mycosis fungoides and Sézary syndrome (SS) account for approximately one third of CTCLs and encompass a heterogenous group of non-Hodgkin lymphomas, ranging from indolent lymphoproliferative disorders to aggressive malignancies with a poor prognosis. The spectrum of CTCLs continues to broaden as new provisional entities are classified. Given the morphologic and histologic overlap among CTCLs and other diagnoses, a thorough clinical history, physical evaluation, and clinicopathologic correlation are essential in the work up and diagnosis of these rare entities. This article will summarize the epidemiologic, clinical, pathologic, and diagnostic features of CTCLs other than mycosis fungoides and SS.

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Key words : adult T-cell lymphoma, adult T-cell leukemia, angioimmunoblastic T-cell lymphoma, CD30⁺ lymphoproliferative disorders, cutaneous T-cell lymphoma, cytotoxic primary cutaneous anaplastic large cell lymphoma, extranodal NK/T-cell lymphoma, nasal type, lymphomatoid papulosis, mycosis fungoides, peripheral T-cell lymphoma, not otherwise specified, 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, Sézary syndrome, subcutaneous panniculitis-like T-cell lymphoma, T-cell lymphoma

Abbreviations used : AECTCL, AITL, ATLL, CAEBV, CTCL, EBV, ENKTL, EORTC, HPS, HTLV-1, HV, LPD, LyP, MF, pcALCL, PCGDTCL, PCSM-TCLPD, PTCL-NOS, SS, SPTCL, TCL, TCR