عنوان

Bone marrow failure /

3319614215

9783319614212

3319614207

9783319614205

Bone marrow failure /

[Book]

Gary M. Kupfer, Gregory H. Reaman, Franklin O. Smith, editors.

Cham, Switzerland :

Springer,

©2018.

1 online resource :

color illustrations

Pediatric oncology

Includes bibliographical references.

Intro; Preface; Acknowledgments; Contents; 1: The Cellular and Molecular Mechanisms of Hematopoiesis; 1.1 Hematopoiesis; 1.2 HSCs; 1.2.1 Transcription Factors; 1.2.2 Epigenetics; 1.2.3 Microenvironment; 1.3 Erythrocyte; 1.3.1 Transcription Factors; 1.3.2 Growth Factor Signaling; 1.3.3 Microenvironment; 1.4 Megakaryocyte; 1.4.1 Transcription Factors; 1.4.2 Growth Factor Signaling; 1.4.3 Microenvironment; 1.5 Monocyte; 1.5.1 Genetic and Epigenetic Regulation; 1.5.2 Growth Factor Signaling; 1.6 Neutrophil; 1.6.1 Transcription Factors; 1.6.2 Growth Factor Signaling.

1.6.3 Microenvironment1.7 Eosinophil; 1.7.1 Genetic; 1.7.2 Growth Factor Signaling; 1.7.3 Microenvironment; 1.8 Basophil and Mast Cells; 1.8.1 Transcription Factors; 1.8.2 Growth Factor Signaling; 1.9 B Cell; 1.9.1 Genetic; 1.9.2 Growth Factor Signaling; 1.9.3 Microenvironment; 1.10 T Cell; 1.11 Natural Killer Cells and Innate Lymphoid Cells; 1.11.1 Transcription Factors; 1.11.2 Growth Factor Signaling; References; 2: Acquired Aplastic Anemia; 2.1 Introduction; 2.2 Epidemiology; 2.3 Etiology; 2.4 Pathophysiology; 2.5 Grading of Severity; 2.6 Clinical Presentation.

2.7 Natural History2.8 Diagnosis and Laboratory Evaluation; 2.9 Treatment; 2.9.1 Supportive Care; 2.9.2 Hematopoietic Stem Cell Transplantation: Matched Sibling Donor Hematopoietic Stem Cell Transplant; 2.9.3 Immunosuppressive Therapy; 2.9.4 Hematopoietic Stem Cell Transplantation: Alternative Donor Hematopoietic Stem Cell Transplant; 2.9.5 Late Effects; 2.9.6 Androgens and Growth Factors; 2.9.7 Myelodysplastic Syndrome and Acute Myeloid Leukemia; 2.9.8 Paroxysmal Nocturnal Hemoglobinuria; References; 3: Pediatric Myelodysplastic Syndromes; 3.1 Introduction.

3.2 Classification of MDS in Pediatric Patients3.2.1 Historical Background; 3.2.2 Differences Between Adult and Pediatric MDS; 3.2.3 Current Classification of Pediatric MDS; 3.3 Refractory Cytopenia of Childhood; 3.3.1 Differentiating Severe Aplastic Anemia from Hypocellular Refractory Cytopenia of Childhood; 3.4 Advanced MDS; 3.5 Challenges of the Current WHO Classification; 3.6 Primary Versus Secondary MDS; 3.7 MDS Due to Germline Predisposition; 3.7.1 MDS Due to Germline Mutations in GATA2; 3.7.2 MDS Due to Germline Mutations in SAMD9; 3.8 Somatic Mutations in MDS.

3.9 Clinical Presentation3.10 Diagnostic Work-Up; 3.10.1 Laboratory Evaluation; 3.10.2 Histopathology; 3.10.3 Cytogenetics and Molecular Studies; 3.10.4 Flow Cytometry; 3.11 Treatment Options in Pediatric MDS; 3.12 Prognosis; References; 4: Fanconi Anemia: A Pathway of Hematopoiesis and Cancer Predisposition; 4.1 Clinical Presentation; 4.2 Diagnosis; 4.3 Acute Myeloid Leukemia/Myelodysplastic Syndrome; 4.4 Solid Tumor; 4.5 Treatment of Patients with Fanconi Anemia; 4.5.1 Androgens; 4.5.2 Hematopoietic Cell Transplantation; 4.5.3 Supportive Care; 4.5.4 Gene Therapy.

0

8

8

8

8

This book presents the latest scientific knowledge on inherited and acquired bone marrow failure syndromes, describing the advances in understanding of genetics and pathophysiology that have been achieved as a result of high-throughput DNA sequencing, RNA expression studies, and modern biochemistry techniques. The full range of relevant conditions is covered, including acquired aplastic anemia, Fanconi anemia, ribosomopathies, telomeropathies, dyskeratosis congenita, Shwachman-Diamond syndrome, Diamond-Blackfan anemia, and congenital neutropenias and thrombocytopenias. In addition, it is explained how the study of these rare diseases has uncovered important new science and elucidated the pathophysiology of more common hematological and oncological diseases. A treatment-specific chapter describes options ranging from the conventional to the cutting edge. Bone Marrow Failure will be of value for both trainee and practicing hematologists and oncologists.

9783319614205

Bone marrow-- Diseases.

Bone marrow-- Diseases.

HEALTH & FITNESS-- Diseases-- General.

MEDICAL-- Clinical Medicine.

MEDICAL-- Diseases.

MEDICAL-- Evidence-Based Medicine.

MEDICAL-- Internal Medicine.

HEA-- 039000

MED-- 014000

MED-- 022000

MED-- 045000

MED-- 112000

MJCL

MJCL

Kupfer, Gary M.

Reaman, Gregory H.

Smith, Franklin O.

20200823102002.0

pn

[Book]

Y