NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) T-Cell Lymphomas Version 2.2019 — December 17, 2018 NCCN.org Continue Version 2.2019, 12/17/18 © 2018 National Comprehensive Cancer Network® (NCCN®), All rights reserved. NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN. NCCN Guidelines Index NCCN Guidelines Version 2.2019 Table of Contents T-Cell Lymphomas Discussion *Steven M. Horwitz, MD/Chair † Þ Bradley M. Haverkos, MD, MPH, MS † Saurabh A. Rajguru, MD † ‡ Memorial Sloan Kettering Cancer Center University of Colorado Cancer Center University of Wisconsin Carbone Cancer Center *Stephen Ansell, MD, PhD/Vice-Chair ‡ Richard T. Hoppe, MD § Mayo Clinic Cancer Center Stanford Cancer Institute Satish Shanbhag, MBBS, MPH † ‡ ξ The Sidney Kimmel Comprehensive Weiyun Z. Ai, MD, PhD † ‡ Eric Jacobsen, MD † Cancer Center at Johns Hopkins UCSF Helen Diller Family Dana-Farber/Brigham and Women's Comprehensive Cancer Center Cancer Center Andrei Shustov, MD † Fred Hutchinson Cancer Research Center/ Jeffrey Barnes, MD, PhD † Deepa Jagadeesh, MD, MPH † ‡ Seattle Cancer Care Alliance Massachusetts General Hospital Case Comprehensive Cancer Center/ Cancer Center University Hospitals Seidman Cancer Center Lubomir Sokol, MD, PhD † ‡ Þ and Cleveland Clinic Taussig Cancer Institute Moffitt Cancer Center Stefan K. Barta, MD, MRCP, MS † ‡ Þ Fox Chase Cancer Center Youn H. Kim, MD ϖ † Pallawi Torka, MD † ‡ Stanford Cancer Institute Roswell Park Cancer Institute Mark W. Clemens, MD ʘ The University of Texas Matthew A. Lunning, DO † Þ ξ Carlos Torres-Cabala, MD ≠ MD Anderson Cancer Center Fred & Pamela Buffett Cancer Center The University of Texas MD Anderson Cancer Center Ahmet Dogan, MD, PhD ≠ Amitkumar Mehta, MD † ‡ Þ Memorial Sloan Kettering Cancer Center University of Alabama at Birmingham Ryan Wilcox, MD, PhD † Comprehensive Cancer Center University of Michigan Francine M. Foss, MD † ‡ ξ Rogel Cancer Center Yale Cancer Center/Smilow Cancer Hospital Neha Mehta-Shah, MD † ‡ Siteman Cancer Center at Barnes- Basem M. William, MD ‡ Aaron M. Goodman, MD ‡ ξ Jewish Hospital and Washington The Ohio State University Comprehensive UC San Diego Moores Cancer Center University School of Medicine Cancer Center - James Cancer Hospital and Solove Research Institute John P. Greer, MD ‡ ξ Elise A. Olsen, MD ϖ † Vanderbilt-Ingram Cancer Center Duke Cancer Institute Jasmine Zain, MD † City of Hope National Medical Center Joan Guitart, MD ≠ ϖ Barbara Pro, MD † Robert H. Lurie Comprehensive Cancer Robert H. Lurie Comprehensive Cancer NCCN Center of Northwestern University Center of Northwestern University Mary Dwyer, MS Hema Sundar, PhD Ahmad Halwani, MD ‡ Huntsman Cancer Institute † Medical oncology Þ Internal medicine at the University of Utah ‡ Hematology/Hematology ϖ Dermatology oncology ʘ Plastic surgery Continue § Radiotherapy/Radiation oncology ¥ Patient advocacy NCCN Guidelines Panel Disclosures ξ Bone marrow transplantation * Discussion Writing Committee ≠ Pathology Member Version 2.2019, 12/17/18 © 2018 National Comprehensive Cancer Network® (NCCN®), All rights reserved. NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN. NCCN Guidelines Index NCCN Guidelines Version 2.2019 Table of Contents T-Cell Lymphomas Discussion NCCN T-Cell Lymphomas Panel Members Summary of the Guidelines Updates Clinical Trials: NCCN believes that the best management for any patient with cancer is in a clinical trial. • Peripheral T-Cell Lymphomas (TCEL-1) Participation in clinical trials is • Breast Implant-Associated ALCL (BIAA-1) especially encouraged. • T-Cell Large Granular Lymphocytic Leukemia (LGLL-1) To find clinical trials online at NCCN • Adult T-Cell Leukemia/Lymphoma (ATLL-1) Member Institutions, click here: • T-Cell Prolymphocytic Leukemia (TPLL-1) nccn.org/clinical_trials/physician.html. • Extranodal NK/T-Cell Lymphoma, nasal type (NKTL-1) NCCN Categories of Evidence and • Hepatosplenic Gamma-Delta T-Cell Lymphoma (HGTL-1) Consensus: All recommendations are category 2A unless otherwise • Principles of Molecular Analysis in T-Cell Lymphomas (LYMP-A) indicated. • Supportive Care (LYMP-B) See NCCN Categories of Evidence • Lugano Response Criteria for Non-Hodgkin’s Lymphoma (LYMP-C) and Consensus. • Principles of Radiation Therapy (LYMP-D) NCCN Categories of Preference: All recommendations are considered • See NCCN Guidelines for Primary Cutaneous Lymphomas appropriate. Primary Cutaneous B-Cell Lymphomas See NCCN Categories of Preference Mycosis Fungoides/Sézary Syndrome Primary Cutaneous CD30+ T-Cell Lymphoproliferative Disorders Classification and Staging (ST-1) Use of Immunophenotyping/Genetic Testing in Differential Diagnosis of Mature B-Cell and NK/T-Cell Neoplasms (See NCCN Guidelines for B-Cell Lymphomas - NHODG-A) The NCCN Guidelines® are a statement of evidence and consensus of the authors regarding their views of currently accepted approaches to treatment. Any clinician seeking to apply or consult the NCCN Guidelines is expected to use independent medical judgment in the context of individual clinical circumstances to determine any patient’s care or treatment. The National Comprehensive Cancer Network® (NCCN®) makes no representations or warranties of any kind regarding their content, use or application and disclaims any responsibility for their application or use in any way. The NCCN Guidelines are copyrighted by National Comprehensive Cancer Network®. All rights reserved. The NCCN Guidelines and the illustrations herein may not be reproduced in any form without the express written permission of NCCN. ©2018. Version 2.2019, 12/17/18 © 2018 National Comprehensive Cancer Network® (NCCN®), All rights reserved. NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN. NCCN Guidelines Index NCCN Guidelines Version 2.2019 Table of Contents T-Cell Lymphomas Discussion Updates in Version 2.2019 of the NCCN Guidelines for T-Cell Lymphomas from Version 1.2019 include: Peripheral T-Cell Lymphomas TCEL-B 1 of 5 • First-line therapy for ALCL The qualifier "ALK+ histology" was removed. "Brentuximab vedotin + CHP (cyclophosphamide, doxorubicin, and prednisone)" was added as a prefrred refimen with a category 1 designation. • First-line therapy for other histologies (PTCL, NOS; AITL; EATL; MEITL; nodal PTCL, TFH; and FTCL) Brentuximab vedotin + CHP (cyclophosphamide, doxorubicin, and prednisone) for CD30+ histologies was added as a preferred regimen with a category 2A designation. Breast Implant-Associated ALCL BIAA-2 • Extended disease (stage II-IV) "Brentuximab vedotin + CHP (cyclophosphamide, doxorubicin, and prednisone)" was added as a systemic therapy option with a category 2A designation. Adult T-Cell Leukemia/Lymphoma ATLL-B 1 of 2 • Initial chemotherapy "Brentuximab vedotin + CHP (cyclophosphamide, doxorubicin, and prednisone) for CD30+ cases" was added as an option with a category 2A designation. Hepatosplenic T-Cell Lymphoma HGTL-4 • Primary treatment "Brentuximab vedotin + CHP (cyclophosphamide, doxorubicin, and prednisone) for CD30+ cases" was added with a category 2A designation. A corresponding footnote was added, "Patients with HGDTL were eligible for the ECHELON-2 study (Horwitz SM, Connor OA, Pro B, et al. The ECHELON-2 trial: results of a randomized, double-blind, active-controlled phase 3 study of brentuximab vedotin and CHP (A+CHP) versus CHOP in the frontline treatment of patients with CD30+ peripheral T-cell lymphomas [abstract]. Blood 2018;132:Abstract 997) but no patients were enrolled." Continued Version 2.2019, 12/17/18 © 2018 National Comprehensive Cancer Network® (NCCN®), All rights reserved. NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN. UPDATES NCCN Guidelines Index NCCN Guidelines Version 2.2019 Table of Contents T-Cell Lymphomas Discussion Updates in Version 1.2019 of the NCCN Guidelines for T-Cell Lymphomas from Version 5.2018 include: New algorithm Peripheral T-Cell Lymphomas • Diagnosis, workup, and treatment recommendations for "Hepatosplenic TCEL-1 T-cell lymphoma (HSTCL)" were added to the Guidelines. (HGTL-1) • Diagnosis, Useful 3rd bullet was revised, "Additional immunohistochemical studies to Global changes characterize subsets of PTCL including markers of T-follicular helper • Suggested treatment regimen references were updated throughout the [TFH] cell origin): ßF1, CXCL13, ICOS, BCL6, TCRdelta, cytotoxic T-cell guidelines. markers (TIA-1, granzyme B, perforin)." • A footnote to the new "See Principles of Molecular Analysis in T-Cell Bullet was removed, "Karyotype to establish clonality." Lymphomas (LYMP-A)" was added to the Diagnosis heading for all subtypes. • A footnote was added to PET/CT scan as appropriate throughout the TCEL-2 guidelines: "Patients with T-cell lymphomas often have extranodal disease, • Workup, Useful which may be inadequately imaged by CT. PET scan may be preferred in "Hepatitis C testing" was added. these instances." • Diagnosis, Useful Under Certain Circumstances, the following bullet and TCEL-3 corresponding footnote were made consistent throughout: "Molecular • ALCL, ALK positive, Stage I, II analysis to detect clonal T-cell antigen receptor (TCR) gene rearrangements ISRT dosing was removed from the page and a footnote with a link to or other assessment of clonality (karyotype, array-CGH, or FISH analysis the