Preface

**Section 3 Benign Hematological Diseases 129**

Vijaya S. Pilli

Ataga

**VI** Contents

**Modulators of Coagulation 131**

Chapter 7 **Understanding the Clotting Cascade, Regulators, and Clinical**

**Presentation, Diagnostic and Therapeutic Challenges 155** Baba P.D. Inusa, Lodi Mariachiara, Palazzi Giovanni and Kenneth I.

Chapter 8 **Sickle Cell Nephropathy: Current Understanding of the**

Chapter 9 **Idiosyncratic Drug-Induced Severe Neutropenia and Agranulocytosis: State of the Art 187** Emmanuel Andrès and Rachel Mourot-Cottet

Hematology has constantly been advancing in parallel with technological developments that have expanded our understanding of the phenotypic, genetic, and molecular complexi‐ ty and extreme clinical and biological heterogeneity of blood diseases. This has in turn al‐ lowed for developing more effective and less toxic alternative therapeutic approaches directed against critical molecular pathways. The continuous and rather extensive influx of new information regarding the key features and underlying mechanisms as well as treat‐ ment options in hematology requires a frequent update of this topic. The primary objective of this book is to provide the specialists involved in the clinical management and experi‐ mental research in hematological diseases with comprehensive and concise information on some important theoretical and practical developments in the biology, clinical assessment, and treatment of patients, as well as on some molecular and pathogenetic mechanisms and the respective translation into novel therapies.

The book comprises two main sections, devoted to hematological malignancies and benign hematological diseases.

**Some major biological mechanisms underlying normal and pathological processes in hematopoietic system** are comprehensively reviewed, e.g., the current concepts of the role of B-cell receptor (BCR) signaling in normal B-cell biology versus B-cell lymphoma patho‐ gens, as well as the advances in the knowledge about the structure, function, and regulation of the coagulation system and the respective defects resulting in hemorrhagic and thrombot‐ ic disorders. The effective implementation of rationally developed therapies requires rapid integration of new biological data.

**Specific nosological entities and clinical scenarios** such as diffuse large B-cell lymphomas, myeloid sarcoma, or sickle cell nephropathy and idiosyncratic drug-induced neutropenia and agranulocytosis are also within the scope of the book. The rapid pace in understanding biological mechanisms, recent molecular subclassification, and clinical developments has moved into the focus of personalization of therapy.

**Innovative laboratory approaches** and the rapid transfer from "laboratory to bedside" are the hallmark of hematology. Currently, classical investigations are integrated with extensively developing high-throughput technologies. Digital pathology integrated with advances in ma‐ chine learning is emerging as a powerful tool to enhance morphology-based decisions. Liquid biopsies emerge as a novel molecular methodology for diagnostics and disease monitoring.

An international panel of experts provides novel insights into various aspects of hematology and contributes their experience to an update of the field. Each chapter is a separate publica‐ tion that reflects each author's views and concepts. However, the book presents an update

and introduces novel insights in novel methodological approaches to study blood, as well as in our current understanding of the biology and clinical presentation, the risk assessment, and therapeutic challenges in patients with hematological diseases.
