Christopher Dittus - Novel Therapeutics for Rare Lymphomas

This Springer imprint is published by the registered company Springer Nature Switzerland AG

The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland

Common diseases have many advantages: a large pool of people to contribute donations, research interest, and significant clinical experience among providers. Conversely, rare diseases have few treatment options, and those they do have are based on limited scientific evidence. Arriving at a diagnosis is often difficult, and many patients are misdiagnosed initially. Only certain academic centers have capabilities to adequately treat rare diseases limiting access to care for the majority of patients with these disorders. Many people are impacted by this because rare diseases, as a whole, affect a large number of individuals. In order to level the playing field, rare diseases need disproportionate attention.

Cancer, taken together, is not rare. In fact, it is the second leading cause of death in the United States (after cardiovascular causes) [1]. This is misleading, though, because cancer is comprised of hundreds of distinct diseases. Few types of cancer exemplify this better than lymphoma, which is broadly divided into Hodgkin and non-Hodgkin lymphoma (NHL), but, in fact, consists of more than 60 unique diseases [2]. Including chronic lymphocytic leukemia (CLL), there are approximately 100,000 cases of lymphoma in the United States each year [3]. Of these, only two groups have over 20,000 cases per year: CLL (20,980 cases in 2016) and diffuse large B-cell lymphoma (DLBCL; 25,380 cases in 2016). None of the remaining subtypes have more than 15,000 cases per year, and most have fewer than 10,000.

In this context, we turn to the focus of this book: novel therapeutics for rare lymphomas. As therapeutic advances race forward for many types of cancer, rare cancers are often lagging behind. New medications are often tested on common cancers to ensure there is ample financial support for the drug. Eventually, a small clinical trial, or often retrospective review, may evaluate the new drug in a rare cancer. Often, one study will make up the entire evidence base for many years. The aim of this book is to highlight research advances in a group of diseases that are often overlooked. This book serves as a single repository of information on the most recent advances in targeted small molecule inhibitors, monoclonal antibodies, and immunotherapy, as they pertain to rare types of lymphoma.

Experts and leaders in the field of lymphoma have been selected for each chapter, and the book will serve as a guide for community oncologists as well as academic oncologists. Each chapter begins with background information on the specific type of lymphoma and then describes standard treatment approaches; the remainder of each chapter focuses on novel treatment approaches.

The reader will find a book that is highly readable, clinically relevant, and easily accessible. The first chapter in the book focuses on hematopathology, which is where all of oncology begins obtaining an accurate diagnosis. This is particularly important for lymphoma, which needs a subtype diagnosis. This can be difficult, even for common types. For rare lymphomas, hematopathology is particularly crucial, as an inexperienced hematopathologist may mistake the diagnosis for a more common lymphoma subtype.

Moving through the book chapters, we focus on both indolent and aggressive B-cell lymphomas, including lymphoplasmacytic lymphoma, plasmablastic lymphoma, mantle cell lymphoma, and primary CNS lymphoma. Certain chapters focus on rare variants of more common types of lymphoma, such as primary mediastinal B-cell lymphoma and TP53-altered chronic lymphocytic leukemia. Additionally, we devote a chapter to an important new treatment approach as it pertains to Hodgkin lymphoma: chimeric antigen receptor (CAR) T-cell therapy.

The latter portion of the book focuses on very rare variants of T-cell lymphomas, including the viral-associated subtypes, adult T-cell leukemia/lymphoma, and extranodal NK/T-cell lymphoma. Other chapters include anaplastic large cell lymphoma, enteropathy-associated T-cell lymphoma, and hepatosplenic T-cell lymphoma. The book concludes with a thorough review of cutaneous T-cell lymphoma.

I hope this book will serve as a reference for practicing oncologists, as well as other interested physicians, residents, and students. It was a great experience bringing together experts and leaders in the field from around the country, and it is my hope that readers will appreciate the depth of knowledge these specialists bring to this volume.