Introduction The understanding of the genetic, epigenetic, immuno- well as for practicing hematologists or oncologists. logical and biological causes of myeloproliferative dis- Each chapter follows a similar architecture and leads orders has substantially improved in the last few years. through epidemiology, genetic and molecular causes, Together with refined tools in pathology, the successful hematological and clinical findings, prognostic factors establishment of mouse models mimicking at least some and current treatment approaches of the diseases. of the myeloproliferative disorders, and murine models Effort has been made to point out the evolving field of novel drugs in this arena but simultaneously diff- allowing to carefully dissect the role of mutations and gene dosage effects of, for example JAK2, this has led to entiate between standard and experimental treatment ever increasing numbers of modified classification approaches. schemes. It is therefore important for the heamtologist Together with the co-editors and all the authors of or oncologist to keep up with this rapid change in classi- the various chapters I hope that the readers of the book fication language, the upcoming of new entities or differ- will enjoy reading and benefit from the information entiation between, or subclassification of, rare diseases provided.

Chronic myeloid disorders 1. Chronic Myeloproliferative Disorders (CMPDs) 1.1. Essential Thrombocythemia (ET) 1.1.1. Autosomal Dominant Familial Essential Thrombocythemia 1.1.2. Philadelphia Chromosome Positive ET and Bcr/Abl Positive ET 1.2. Polycythemia Vera (PV) 1.2.1. Autosomal dominant Familial PV 1.3. Melofibrosis with Myeloid Metaplasia (MMM) 1.3.1. Agnogenic Myeloid Metaplasia (AMM) or Chronic Idiopathic Myelofibrosis (CIMF 32) 1.3.2. Atypical Variants 1.3.3. Secondary MF 2. Myelodysplastic Syndroms 2.1. MDS-variants 2.1.1. Therapy-related MDS 2.1.2. Hypocellular MDS 2.1.3. Hyperfibrotic MDS 2.1.4. 5p-Syndrome 2.1.5. 17p-Syndrome 2.1.6. Monosomy 7 Syndrome 2.1.7. Trisomy 8 2.1.8. 3q21q26 Syndrome 2.1.9. Overlap Syndromes 2.1.9.1. Paroxysmal nocturnal haemoglobinuria (PNH) and MDS/PNH 2.1.9.2. Aplastic anemia (AA) and AA/PNH 2.1.9.3. Large granulocytic lymphoma (LGL) and MDS/LGL 3. Atypical Chronic Myeloid Disorders 3.1. CMML 3.2. Atypical CML 3.3. Chronic Neutrophilic Leukemia 3.4. Mast Cell Leukemia 3.5. Chronic Eosinophilic Leukemia 3.6. Chronic Myelogenous Leukemia 3.6.1. Atypical CML

Introduction The understanding of the genetic, epigenetic, immuno- well as for practicing hematologists or oncologists. logical and biological causes of myeloproliferative dis- Each chapter follows a similar architecture and leads orders has substantially improved in the last few years. through epidemiology, genetic and molecular causes, Together with refined tools in pathology, the successful hematological and clinical findings, prognostic factors establishment of mouse models mimicking at least some and current treatment approaches of the diseases. of the myeloproliferative disorders, and murine models Effort has been made to point out the evolving field of novel drugs in this arena but simultaneously diff- allowing to carefully dissect the role of mutations and gene dosage effects of, for example JAK2, this has led to entiate between standard and experimental treatment ever increasing numbers of modified classification approaches. schemes. It is therefore important for the heamtologist Together with the co-editors and all the authors of or oncologist to keep up with this rapid change in classi- the various chapters I hope that the readers of the book fication language, the upcoming of new entities or differ- will enjoy reading and benefit from the information entiation between, or subclassification of, rare diseases provided.