Meet the editor

Ota Fuchs graduated from the Chemical Technological University, Prague, Czech Republic, in 1971. He obtained his PhD in Biochemistry from the Faculty of Natural Sciences, Charles University, Prague, in 1981. He is employed as a senior scientist at the Institute of Hematology and Blood Transfusion, Prague. He undertook a visiting scientist short-term affiliation in the Beatson Institute for Cancer Research, Glasgow, UK, the Institute of

Experimental Medicine of the Russian Academy of Medical Sciences in St. Petersburg, Russia, and in the Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Canada. He was the principal investigator of five projects for the Internal Grant Agency of the Ministry of Health of the Czech Republic and of one grant project for the Grant Agency of the Czech Republic. He is a member of the Czech Medical Society J.E. Purkyne and the Czech MDS Group.

**Preface III**

**Chapter 1 1**

**Chapter 2 13**

**Chapter 3 31** Myelodysplastic Syndrome and Autoimmune Disorders: Causal Relationship or

**Chapter 4 45**

**Chapter 5 63** Myelodysplastic Syndromes: An Update on Pathophysiology and Management

**Chapter 6 85**

*by Andrea Hruštincová, Katarina Szikszai, Zdeněk Krejčík, Nikoleta Loudová* 

Immune Dysregulation in MDS: The Role of Cytokines and Immune Cells

Introductory Chapter: Progress in Myelodysplastic Syndrome Area

Diagnosis and Classification of Myelodysplastic Syndrome *by Gamal Abdul Hamid, Abdul Wahab Al-Nehmi and Safa Shukry*

*by Kam A. Newman, Mojtaba Akhtari and Sheda Heidarian*

*by Selma D'Silva, Sunil B. Rajadhyaksha and Meenakshi Singh*

*by Wanxing Chai-Ho and Gary J. Schiller*

*and Michaela Dostálová Merkerová*

Noncoding RNAs in Myelodysplastic Syndromes

*by Ota Fuchs*

Contents

Coincidence?

## Contents


Preface

Genome and exome sequencing together with targeted deep-sequencing studies have defined several gene mutations in almost every myelodysplastic syndrome (MDS) patient. These mutations can impact disease phenotype, prognosis and disease progression. However, there are many non-pathogenic mutations, and most mutations are not specific for MDS. Applying this information clinically is in progress even if the clinical impact of some mutations remains controversial. Several mutations will likely be incorporated into future prognostic scoring systems. Great progress has also been reached in the studies of non-coding RNAs in MDS. It is possible that they will be used in MDS diagnostics and prognosis. Big advancements in the studies of immune mechanisms in MDS have been achieved and translated into clinical studies of immunotherapies in MDS. Innate and adaptive immune pathways are excessively active in the niche of hematopoietic cells in MDS. Common etiological mechanisms of MDS and autoimmune diseases is possible because these diseases are often associated with MDS. Unfortunately, no new drugs have been approved for MDS since approval by the Food and Drug Administration of azacitidine in 2004, lenalidomide in 2005, and decitabine in 2006. The European Medicines Agency approved azacitidine in 2008 and lenalidomide in 2013. This progress in many aspects of MDS warrants this new book about this heterogeneous group of clonal neoplasms arising from hematopoietic stem cells, and characterized by inefficient hematopoiesis, peripheral cytopenias, frequent karyotypic abnormalities, risk of clonal evolution, and transformation to acute myeloid leukemia, three years

after the last book about MDS published by IntechOpen.

This book provides a review of several fields of MDS not only for research workers and clinicians, but also for medical students with an interest in MDS. The first introductory chapter deals with recent progress in this area. The second chapter provides a review of diagnosis and classification of MDS. Chapter 3 discusses the association of MDS and autoimmune disorders. Chapter 4 introduces immune dysregulation in MDS. The fifth chapter analyzes an update on pathophysiology and

management of MDS. The last chapter discusses non-coding RNAs in MDS.

**Ota Fuchs, PhD**

Department of Genomics, Prague, Czech Republic

Institute of Hematology and Blood Transfusion,
