Color Atlas of Clinical Hematology

Molecular and Cellular Basis of Disease
 
 
Standards Information Network (Verlag)
  • 5. Auflage
  • |
  • erschienen am 21. November 2018
  • |
  • 600 Seiten
 
E-Book | ePUB mit Adobe-DRM | Systemvoraussetzungen
978-1-119-17064-8 (ISBN)
 
Provides coverage of the pathogenesis, clinical, morphologic, molecular and investigational aspects of a full range of blood disorders seen in daily practice

The revised fifth edition of this renowned atlas presents readers with a comprehensive, visual guide to clinical hematology, featuring 2700 full-color photographs and figures depicting the spectrum of hematological diseases. Ranging from photographs of the clinical manifestations and key microscopic findings to diagrams of the molecular aspects of these diseases, the book provides up-to-date information of the blood diseases that clinicians encounter every day.

Color Atlas of Clinical Hematology: Molecular and Cellular Basis of Disease offers the reader an understanding of normal cell machinery, and of the molecular basis for such processes as DNA and cell replication, RNA species, trafficking and splicing, protein synthesis, transcription factors, growth factor signal transduction, epigenetics, cell differentiation, autophagy, and apoptosis. The text goes on to explore how these processes are disturbed in the various diseases of the bone marrow, blood, and lymphoid systems.



Helps solve difficult diagnostic challenges and covers complex principles using highly illustrative, full-color images
Explores all aspects of benign and malignant hematology, including blood transfusion and coagulation with extensive coverage of the pathogenesis of common clinical entities
Provides a quick and easy reference of key diagnostic issues in a comprehensive yet concise format
Includes and illustrates the WHO Classification of Hematologic Malignancies
Illustrates the new knowledge of the molecular basis of inherited and acquired blood diseases

Color Atlas of Clinical Hematology: Molecular and Cellular Basis of Disease is the must-have resource for both trainee and practising hematologists, and for every department of hematology.

"Substantially updated and now multi-authored so that all aspects of haematology are equally covered, including the newest developments in molecular biology and genomic sequencing"

"There is a surplus of invention in communicating complex problems here and an admirable effort to keep the reader totally up-to-date"
5th Revised edition
  • Englisch
  • USA
John Wiley & Sons Inc
  • Für höhere Schule und Studium
  • Überarbeitete Ausgabe
  • Reflowable
  • 268,76 MB
978-1-119-17064-8 (9781119170648)

weitere Ausgaben werden ermittelt
A. Victor Hoffbrand, MA, DM, FRCP, FRCPath, DSc, FMed Sci, is Emeritus Professor of Haematology at University College, London, UK.

Paresh Vyas, FRCP, FRCPath, DPhil, is Professor of Haematology, Director of Oxford Centre for Haematology, Honorary Consultant Haematologist and Group Leader, MRC Molecular Haematology Unit, Radcliffe Department of Medicine, Weatherall Institute of Molecular Medicine, University of Oxford, Oxford, UK.

Elias Campo, MD, is Senior Consultant and Research Director, Hospital Clinic of Barcelona, and Professor of Anatomical Pathology at University of Barcelona, Faculty of Medicine, Barcelona, Spain.

Torsten Haferlach, MD, MLL Munich Leukemia Laboratory, Munich, Germany.

Keith Gomez, MBBS, MRCP, FRCPath, PhD, is Consultant Haematologist at Royal Free London NHS Foundation Trust, UK.
Preface xxx

1 Molecular Biology of the Cell

2 Hematopoiesis

3 Growth Factor Signaling

4 Erythropoiesis and Examination of the Peripheral Blood and Bone Marrow

5 Hypochromic Anemias

6 The Porphyrias and Iron Overload

7 Megaloblastic Anemias

8 Hemolytic Anemias

9 Genetic Disorders of Hemoglobin

10 Benign Disorders of Phagocytes

11 Benign Disorders of Lymphocytes and Plasma Cells

12 Aplastic and Dyserythropoietic Anemias

13 The Hematologic Neoplasms: Laboratory Techniques and Acute Myeloid Leukemia

14 Acute Lymphoblastic Leukemia

15 Myelodysplastic Syndromes

16 Myeloproliferative Neoplasms

17 Mastocytosis, Myeloid/Lymphoid Neoplasms with Eosinophilia and Specific Cytogenetic Rearrangements, Myelodysplastic/Myeloproliferative Neoplasms

18 Chronic Lymphocytic Leukemia and Other Mature B- and T/Nk-Cell Leukemias

19 Small B-Cell Lymphomas

20 Aggressive Mature B-Cell Neoplasms

21 Myeloma and Related Neoplasms

22 Peripheral T- and NK-Cell Neoplasms

23 Hodgkin Lymphoma

24 Histiocytic Disorders

25 Stem Cell Transplantation

26 Normal Hemostasis, Platelet Production and Function

27 Vascular and Platelet Bleeding Disorders

28 Inherited and Acquired Coagulation Disorders

29 Thrombosis and Antithrombotic Therapy

30 Hematologic Aspects of Systemic Diseases

31 Parasitic Disorders

32 Blood Transfusion

Appendix 1 2016 World Health Organization Classification of Lymphoid and Myeloid Neoplasms

Index xxx

CHAPTER 1
MOLECULAR BIOLOGY OF THE CELL


The aim of the first chapter is to provide a primer covering our understanding of the basic molecular and cellular processes of the cell, which inform a scientific understanding of hematologic diseases.

COMPARTMENTALIZATION OF THE CELL


A central evolutionary advance was the compartmentalization of cells, as shown in Fig. 1.1. The cell is bounded by a complex cell membrane that allows regulation of molecules into and out of the cell. Within the cytoplasm a number of different organelles perform key functions. For example, as described later in this chapter, mitochondria are critical for adenosine triphosphate (ATP) generation and heme biosynthesis. Proteins are translated from amino acids and undergo post-translational modification in the Golgi complex and rough endoplasmic reticulum. Depending on the cell type, there are specialized structures within the cytoplasm that allow the cell to perform its specialized role.

Fig. 1.1. A, Photomicrograph showing the morphology of many cells with prominent nucleoli, in this case, B cells. B, A schematic representation of the intracellular composition as visualized by electron microscopy. The nucleus is composed of euchromatin, which is less condensed, paler, and more transcriptionally active, and heterochromatin, which is more condensed, darker, and less transcriptionally active. In cytoplasm subcellular organelles including mitochondria, rough endoplasmic reticulum, and the Golgi complex are shown. The function of these organelles is discussed later.

(Courtesy of Professor JV Melo.)

THE NUCLEUS


As we focus in on the nucleus, it is clear that it is also bounded by a specialized nuclear envelope and membrane (Fig. 1.2). Entry and exit out of the nucleus is regulated by nuclear pores. Within the nucleus, deoxyribonucleic acid (DNA) is tightly packaged by proteins and the DNA/protein complex is known as chromatin. Chromatin has different appearances under light or electron microscopes. When DNA is tightly packaged (and the genes more likely to be not expressed), it is known as heterochromatin. Under the light/electron microscope it appears darker. When DNA is less tightly packaged it is called euchromatin and is lighter in appearance. The other visible structure within the nucleus, in some cells, is the nucleolus, where ribosomal genes are transcribed and assembly of the ribosome takes place (as discussed later).

Fig. 1.2. Schematic representation of a portion of the nucleus. The nucleus is highly compartmentalized, containing specialized structures. The nucleolus is composed of a pars granulosa, a pars fibrosa, and a nucleolar organizing center and makes transfer RNA. The nucleus is bounded by a nuclear envelope that is lined by rough endoplasmic reticulum. There is controlled entry and exit into the nucleus via nuclear pores.

The DNA in the nucleus is distributed among 22 pairs of autosomal chromosomes (numbered 1-22, in order of size) and two sex chromosomes (Fig. 1.3A). When cells are in the metaphase phase of the cell cycle, chromosomes condense and can be visualized by a technique called karyotyping. Chromosomes are divided into two arms: a short arm, termed p, and a longer arm, q. The region where the chromosomes join is termed the centromere. Chromosomes are further subdivided into light and dark bands (depending on how they stain with the Giemsa dye) (Fig. 1.3B). When cells are not in metaphase, chromosomes are more diffusely spread through the nucleus. Most current evidence suggests that the chromosomes occupy discrete territories (chromosomal territories) within a nucleus (Fig. 1.3C). These territories need not be contiguous and can be shared with other chromosomes. However, there are still many aspects of how chromosomes are organized that remain unclear. For example, what constrains chromosomes to territories and how do territories affect gene regulation? Recent work suggests that within chromosomal territories chromatin exists in topologically associated domains (TADs) and that actively expressed genes along the chromosome and possibly even from different chromosomes may congregate in specialized structures where RNA is made from (transcribed) from genes. This process is called transcription and the specialized structures are known as transcription factories (see later).

Fig. 1.3. A, DNA in the human nucleus is organized into 46 chromosomes. There are two copies of chromosomes 1-22 with two sex chromosomes (XX or XY). Each chromosome is divided into a short arm (p) and a long arm (q) and then subdivided into major numeric subsections. For example, the short arm of chromosome 1 (1p) has three subsections and the long arm (1q) has four subsections. B, The gross subdivision of chromosome can be visualized by Giemsa staining of chromosomes that have been subject to brief proteolytic cleavage. (B, Courtesy of Professor H Lodish.) C, Within an interphase nucleus chromosomes occupy discrete territories. The figure shows the territory occupied by chromosome 11 (red color) in a primary erythroblast.

(C, Courtesy of Jo Green and Dr. Veronica Buckle.)

The sequencing of the human genome was a landmark in biology. It allowed all the human genes arrayed along the chromosomes to be catalogued (Table 1.1). Genes are divided into protein-coding genes (of which there are ~21?000), genes that encode different types of RNA (e.g. ribosomal RNA, micro-RNAs, small nuclear RNA), and RNA moieties that are not translated into a functional protein or RNA (pseudogenes). The genome also dedicates sequence to other RNA species that do not make protein but that regulate either transcription or the production of protein from RNA (a process known as translation). These RNA sequences include micro-RNAs, long and short noncoding RNAs. There are also sequences dedicated to regulating transcription of individual genes or banks of genes; these are called promoters and enhancers. This provides a primary description of our genetic makeup. The characterization of the human genome is still being refined as we understand more about how genes are organized and how transcriptional expression and protein translation is controlled.

TABLE 1.1. ALL THE GENES AND OPEN READING FRAMES IN THE HUMAN GENOME HAVE BEEN CHARACTERIZED FROM THE SEQUENCING OF THE HUMAN GENOME. THIS TABLE SHOWS THE SIZE OF EACH CHROMOSOME (IN MEGABASES) AND NUMBER OF GENES AND PSEUDOGENES ON EACH CHROMOSOME.

        Chromosome number Size (Mb) Gene Pseudogene 1 248.96 5,078 1,372 2 242.19 3,862 1,166 3 198.3 2,971 887 4 190.22 2,441 799 5 181.54 2,578 766 6 170.81 3,000 876 7 159.35 2,774 896 8 145.14 2,152 661 9 138.4 2,262 702 10 133.8 2,174 631 11 135.09 2,920 835 12 133.28 2,521 680 13 114.36 1,381 477 14 107.04 2,055 583 15 101.99 1,814 555 16 90.34 1,920 451 17 83.26 2,432 541 18 80.37 988 295 19 58.62 2,481 514 20 64.44 1,349 329 21 46.71 756 202 22 50.82 1,172 348 X 156.04 2,158 859 Y 57.23 577 395 MT 0.016569 37 -

Genes themselves are composed of DNA, which is made up of four nucleotides. Each nucleotide consists of a phosphate group linked by a phosphoester bond to a pentose sugar molecule (ribose) that lacks a hydroxyl group (thus it is deoxyribose), which is then attached to one of four heterocyclic carbon- and nitrogen-containing organic rings: adenine (A), cytosine (C), guanine (G) and thymidine (T). C and T are known as pyrimidines and A and G as purines. These are then linked together into polynucleotides via phosphoester bonds. As James Watson and Francis Crick correctly proposed, these are organized into two associated antiparallel polynucleotide strands that have a 5´ to 3´ direction and form a double helix. The strands are held in register by...

"Substantially updated and now multi-authored so that all aspects of haematology are equally covered, including the newest developments in molecular biology and genomic sequencing. The latter is perhaps best realized in the first chapter 'Molecular Biology of the Cell', where the text and superb figures merge into a highly readable crash course in molecular biology.....I particularly enjoyed the chapter on myelodysplastic syndromes, where clonal heterogeneity is now did actically illustrated.....There is a surplus of invention in communicating complex problems here and an admirable effort to keep the reader totally up-to-date. Of note, there is now an eTextbook version of the Atlas.....Whenever there comes a new model of your smartphone, reviews always try to decide whether you should update from your old model. I would say that there is every reason to update your atlas, even if you have the second last edition. In conclusion, should you encounter one of the knowledge hungry students (be they of medical or master of science background) asking you whether haematology is an exciting career path to embark upon, one way to answer the question would be to hand her/him this atlas for half an hour. It is a major accomplishment and an excellent recruiting tool!"- Peter Hokland (British Journal of Haematology)

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