Understanding Non-Hodgkin Lymphoma A Guide for Patients, Survivors, and Loved Ones July 2018 Lymphoma Research Foundation (LRF) Helpline and Clinical Trials Information Service CONTACT THE LRF HELPLINE Trained staff are available to answer questions and provide support to patients, caregivers and healthcare professionals in any language. Our support services include: • Information on lymphoma, treatment options, side effect management and current research fi ndings • Financial assistance for eligible patients and referrals for additional fi nancial, legal and insurance help • Clinical trial searches based on patient’s diagnosis and treatment history • Support through LRF’s Lymphoma Support Network, a national one-to one volunteer patient peer program Monday through Friday, Toll-Free (800) 500-9976 or email [email protected] Understanding Non-Hodgkin Lymphoma A Guide For Patients, Survivors, and Loved Ones July 2018 This guide is an educational resource compiled by the Lymphoma Research Foundation to provide general information on adult non- Hodgkin lymphoma. Publication of this information is not intended to replace individualized medical care or the advice of a patient’s doctor. Patients are strongly encouraged to talk to their doctors for complete information on how their disease should be diagnosed, treated, and followed. Before starting treatment, patients should discuss the potential benefits and side effects of cancer therapies with their physician. Contact the Lymphoma Research Foundation Helpline: (800) 500-9976 [email protected] Website: lymphoma.org This patient guide is supported through unrestricted educational grants from: © 2018 Lymphoma Research Foundation. Information contained herein is the property of the Lymphoma Research Foundation (LRF). Any portion may be reprinted or reproduced provided that LRF is acknowledged to be the source and the Foundation’s website (lymphoma.org) is included in the citation. ACKNOWLEDGMENTS The Lymphoma Research Foundation wishes to acknowledge those individuals listed below who have given generously of their time and expertise. We thank them for their contributions, editorial input, and professional knowledge, which have truly enhanced this publication. The review committee guided the content and development of this publication. Without their dedication and efforts, this publication would not have been possible. We hope those in the lymphoma and chronic lymphocytic leukemia community will now be better informed and have a better understanding of these diagnoses because of the gracious efforts of those involved in the planning and execution of this comprehensive disease guide. Review Committee Editorial Board Steering Committee Editorial Chairman Morton Coleman, MD, NewYork-Presbyterian Hospital/Weill Cornell Medicine Medical Review Board Jorge J. Castillo, MD, Dana-Farber Cancer Institute/Harvard Medical School Bruce D. Cheson, MD, FACP, FAAAS, FASCO, Lombardi Comprehensive Cancer Center, Georgetown University Hospital Kieron M. Dunleavy, MD, GW Cancer Center, George Washington University Arnold S. Freedman, MD, Dana-Farber Cancer Institute Jonathan W. Friedberg, MD, MMSc, James P. Wilmot Cancer Institute, University of Rochester Medical Center Leo I. Gordon, MD, FACP, Robert H. Lurie Comprehensive Cancer Center of Northwestern University ii Understanding Non-Hodgkin Lymphoma Thomas M. Habermann, MD, Mayo Clinic, Rochester Brad S. Kahl, MD, Washington University Medical School John P. Leonard, MD, NewYork-Presbyterian Hospital/Weill Cornell Medicine John M. Pagel, MD, PhD, Swedish Cancer Institute Lauren C. Pinter-Brown, MD, FACP, Chao Family Comprehensive Cancer Center, University of California at Irvine Steven T. Rosen, MD, City of Hope Sonali M. Smith, MD, The University of Chicago Medicine Eduardo M. Sotomayor, MD, GW Cancer Center, George Washington University Michael E. Williams, MD, ScM, University of Virginia Cancer Center Andrew D. Zelenetz, MD, PhD, Memorial Sloan Kettering Cancer Center Lymphoma Research Foundation Reviewers Izumi Nakano, Associate Director of Patient Services Peggy Ann Torney, Chief Strategy, Communications and Engagement Officer Understanding Non-Hodgkin Lymphoma iii TABLE OF CONTENTS Abbreviations ..........................................4 Introduction ............................................6 Part 1 — Learning the Basics .........................7 Chapter 1: Understanding the Disease ...................7 Table 1.1. Main Types of Indolent and Aggressive NHLs (Listed Alphabetically) ..............................16 Chapter 2: Seeking Medical Attention ...................29 Table 2.1. Signs and Symptoms Commonly Found in Patients With NHL .................................30 Chapter 3: Receiving a Diagnosis ......................33 Table 3.1. The Three Main Types of Biopsies ............35 Table 3.2. Immunohistochemistry and Flow Cytometry Tests ..............................38 Chapter 4: Work-Up Before Treatment Can Begin .........42 Table 4.1 The Eastern Cooperative Oncology Group Performance Status Scale ...........................43 Table 4.2. Types of Imaging Tests .....................45 Table 4.3. Other Types of Biopsies ....................46 1 Understanding Non-Hodgkin Lymphoma Part 2 — Treatment of Non-Hodgkin Lymphoma .....52 Chapter 5: What to Know Before Starting Treatment ......52 Table 5.1. International Prognostic Index ...............55 Chapter 6: Treatments for Non-Hodgkin Lymphoma .......66 Table 6.1. Common Chemotherapy Regimens for NHL ...69 Table 6.2. Catheters Used to Administer Chemotherapy. 72 Table 6.3. Methods for Delivering Radiation Therapy ....88 Table 6.4. Terms Used to Describe Treatment and Its Outcomes ..................................94 Table 6.5. Forms of Complementary Therapy ...........97 Part 3 — Survivorship and Living With the Side Effects of Treatment. 99 Chapter 7: Common Treatment Side Effects .............99 Table 7.1. Five Common Conditions Caused by Decreased Blood Cell Production ...................102 Chapter 8: Managing Life During and After Treatment ....127 Table 8.1. Coping Strategies ........................127 Understanding Non-Hodgkin Lymphoma 2 Part 4 — Hospital Admission .......................133 Chapter 9: Preparing to Go to the Hospital ..............133 Part 5 — Clinical Trials and Advances in Treatment ........................................140 Chapter 10: Overview of Clinical Trials .................140 Table 10.1. The Four Phases of Clinical Trials .......... 141 Chapter 11: Advances in Treatment of Patients With Non-Hodgkin Lymphoma ............................146 About The Lymphoma Research Foundation ..............150 3 Understanding Non-Hodgkin Lymphoma ABBREVIATIONS 3D-CRT three-dimensional CT computed tomography conformal radiation CTCL cutaneous T-cell therapy lymphoma ABC activated B-cell–like DHL double-hit lymphoma ABMS American Board of DLBCL diffuse large B-cell Medical Specialties lymphoma AIDS acquired DLCO diffusing capacity of immunodeficiency the lungs for carbon syndrome monoxide AIHA autoimmune hemolytic DNA deoxyribonucleic acid; anemia genetic material AITL angioimmunoblastic DNR do not resuscitate T-cell lymphoma EATL enteropathy-associated ALCL anaplastic large cell T-cell lymphoma lymphoma EBV Epstein-Barr virus ALK anaplastic lymphoma ECHO echocardiogram kinase ENMZL extranodal marginal zone ALL acute lymphoblastic lymphoma leukemia FDA U.S. Food and Drug ANC absolute neutrophil count Administration ASCO American Society of FISH fluorescence in situ Clinical Oncology hybridization ASH American Society of FL follicular lymphoma Hematology FNA fine needle aspiration B2M beta-2 microglobulin GCB germinal center B-cell– BTK Bruton tyrosine kinase like CAR chimeric antigen receptor GVHD graft-versus-host disease CBC complete blood count GVL graft-versus-lymphoma CLL chronic lymphocytic HBV hepatitis B virus leukemia HCV hepatitis C virus CNS central nervous system HDAC histone deacetylase CPR cardiopulmonary HIV human resuscitation immunodeficiency virus CR complete remission HL Hodgkin lymphoma CSF cerebrospinal fluid Abbreviations 4 HTLV-1 human T-cell PET positron emission lymphotropic virus type 1 tomography IgM immunoglobulin M PFT pulmonary function test IGRT image-guided radiation PI3K phosphoinositide (or therapy phosphatidylinositol)-3- IHC immunohistochemistry kinase IMiD immunomodulatory drug PICC peripherally inserted IPI International Prognostic central catheter Index PMBCL primary mediastinal IRB institutional review board B-cell lymphoma ITP immune PML progressive multifocal thrombocytopenia leukoencephalopathy IV intravenous PR partial remission LDH lactate dehydrogenase PS performance status LRF Lymphoma Research PTCL peripheral T-cell Foundation lymphoma MALT mucosa-associated PTCL- lymphoid tissue NOS peripheral T-cell MCL mantle cell lymphoma lymphoma, not otherwise MMAE monomethyl auristatin E specified MR minor response RNA ribonucleic acid; genetic MRI magnetic resonance material imaging SAB scientific advisory board MUGA multigated acquisition SEER Surveillance, MZL marginal zone lymphoma Epidemiology, and End NCCN National Comprehensive Results Cancer Network SLL small lymphocytic NCI National Cancer Institute lymphoma NHL non-Hodgkin lymphoma SMZL splenic marginal zone NIH National Institutes of lymphoma Health Syk spleen tyrosine kinase NK natural killer (cell) TLS tumor lysis syndrome NSAID nonsteroidal anti- TNF tumor necrosis factor inflammatory drug TSEBT total skin electron beam PCR polymerase chain therapy reaction