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 course, prognoses, and management. With 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 where staging is recommended, Ann Arbor or TNM 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 including multiagent 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.
Yuna Oh, Joseph R Stoll, Alison Moskowitz, Melissa Pulitzer, Steven Horwitz, Patricia Myskowski, Sarah J Noor