Meet the editor

Pier Paolo Piccaluga is currently Associate Professor of Pathology at the Department of Experimental, Diagnostic and Specialty Medicine, Bologna University School of Medicine—Institute of Hematology and Medical Oncology, and has been responsible for many years for the Molecular Pathology Laboratory. In 2018 he was appointed for teaching at Queen Mary University of London (UK) and Jomo Kenyatta University of Agriculture and Technol-

ogy, Nairobi (Kenya). He is the author of several international publications indexed in the Current Contents (215 publications quoted in PubMed, including *Nature Medicine*, *Journal of Clinical Investigation*, *Journal of Experimental Medicine*, *Journal of Clinical Oncology*, *Blood*, *Lancet Oncology*, and *Lancet Infectious Diseases*, with a total impact factor >1,000, a mean impact factor >6, and an H-index of 51 (ranked among the VIA-Academy Top Italian Scientists).

Contents

*by Pier Paolo Piccaluga*

*by Suzanne D. Turner*

*by Silvana Novelli*

*by Philipp Staber*

in T-cell Lymphoma

Overview of T-cell Lymphomas

Anaplastic Large Cell Lymphoma

Extranodal T/NK Lymphomas

**Preface III**

**Chapter 1 1**

**Chapter 2 7**

**Chapter 3 21**

**Chapter 4 43**

**Chapter 5 55**

**Chapter 6 77**

**Chapter 7 89**

**Chapter 8 107**

Unique Therapeutic Approaches for Targeting Epigenetic Machinery

Novel Aurora Kinase Inhibitor-Based Combination Therapies for PTCL

Introductory Chapter: Updates and New Insights from WHO 2017

Peripheral T-Cell Lymphoma Classification

*by Nagavalli Somasundaram and Soon Thye Lim*

Gamma-Delta T-cell Lymphoma: An Overview

Precision Medicine Concepts in T-Cell Lymphoma

*by Pavan Tenneti, Lisa E. Davis and Daruka Mahadevan*

*by Jacob Cogan and Jennifer E. Amengual*

*by Preethi Ramachandran, Alok Aggarwal and Jen Chin Wang*

## Contents


Preface

Tumors derived from T-lymphocytes are uncommon, representing 10% of lymphoid malignancies overall and encompassing several extremely rare different entities. However, they represent an important diagnostic and therapeutic challenge. In fact, on the one hand, their rarity, heterogeneity, as well as the lack of specific diagnostic markers make their diagnosis quite difficult. Indeed, large studies performed in Europe and the United States showed that up to 30% of cases are misdiagnosed. On the other hand, largely due to their rarity, the currently used therapeutic schemes are basically derived from those adopted for B-cell malignancies and appear definitely unsatisfactory. Only recently have specific clinical trials been dedicated to T-cell lymphomas, leading to the approval of a few new drugs for second-line treatments. Hopefully, currently ongoing trials will be able to show some benefit when such new drugs are included in first-line

In this book, a brief overview on the most updated World Health Organization (WHO) classification and the general features of peripheral T-cell lymphomas (PTCLs) is first provided. Thereafter, a few entities have been described in more detail and, eventually, new concepts of personalized targeted treatments are listed and discussed. A particular emphasis has been given to anaplastic large cell lymphomas, the classification of which has been significantly modified in the new WHO blue book, now including anaplastic lymphoma kinase-positive and -negative forms, as well as the cutaneous and breast implant-associated types. Similarly, non-cutaneous and gamma-delta T-cell lymphomas has been described according to their new classification. In all sections and chapters, the role of molecular genetics has been considered, highlighting its role in the development of novel classification as well as of new, more rational targeted approaches. In fact, the better understanding of PTCL pathophysiology, largely based on gene expression profiling and next-generation sequencing studies, had a pivotal role in the definition of novel targets for more effective therapies. As prototypic examples of this process, therapies targeting the epigenetic machinery as well as Aurora kinase inhibitors have been deeply described. It should not be forgotten, nonetheless, that the prognosis of PTCL patients is still generally dismal and extensive basic and clinical research have to be carried out in the future to improve the present

The book is intended for all health professionals, particularly for those involved in the diagnosis and treatment of oncological diseases, as well as medical students and

approaches as well.

scenario.

fellows.
