Williams Hematology: The Red Cell and Its Diseases

by Josef T. Prchal, Marshall A. Lichtman

Overview

Everything you need to know about red cell disorders, from basic science to clinical application.

When red blood cells do not have enough hemoglobin, oxygen cannot reach all parts of the body—as a result, organs start to malfunction and may lead to a host of issues including deformities, enlarged spleen, heart problems and other diseases. There are myriad red cell disorders, such as iron deficiency anemia, pernicious anemia, aplastic anemia, autoimmune hemolytic anemia, sickle cell, thalassemia and other hemoglobinopathies.

The Red Cells and its Diseases provides clinicians with a practical diagnostic and treatment framework for identifying and successfully managing acute, congenital, and chronic red blood cell disorders. Presented in an accessible template format, the book includes classifications, etiology and pathogenesis, clinical and laboratory features, differential diagnosis, treatment, management, and supportive care.

This comprehensive, yet concise resource covers:

• Structure and physiology of the red cell
• Classification of red cell diseases
• Diseases of red cell production
• Anemias resulting principally from inherited disorders
• Anemia as a result of exogenous factors
• Polyclonal erythrocytosis
• Red cell transfusion

Product Details

ISBN-13:	9781264269075
Publisher:	McGraw Hill LLC
Publication date:	11/05/2021
Pages:	608
Product dimensions:	5.90(w) x 9.00(h) x 0.90(d)

About the Author

Marshall A. Lichtman, MD, is dean emeritus, and a former professor of medicine, biochemistry, and biophysics at the University of Rochester Medical Center in New York. He is a past president of the American Society of Hematology (ASH).

Josef T. Prchal, MD, is a professor in the division of hematology and hematologic malignancies, a Huntsman Cancer Institute investigator, and a member of the Cell Response and Regulation Program and the Imaging, Diagnostics, and Therapeutics Program at the University of Utah.

Marshall Lichtman, M.D.Professor of MedicineUniversity of Rochester School of MedicineRochester, NY

Table of Contents

Contributors ix

Preface xiii

Part I

Structure and Physiology of the Red Cell

1 Structure and Composition of the Erythrocyte Mohandas Narla 3

2 Erythropoiesis and Red Cell Turnover Josef T. Prchal Perumal Thiagarajan 21

Part II

Classification of Red Cell Diseases

3 Clinical Manifestations and Classification of Erythrocyte Disorders Josef T. Prchal 49

Part III

Diseases of Red Cell Production

4 Aplastic Anemia: Acquired and Inherited George B. Segel Marshall A. Lichtman 61

5 Pure Red Cell Aplasia Neal S. Young 91

6 Anemia of Chronic Disease Tomas Ganz 101

7 Erythropoietic Effects of Endocrine Disorders Xylina T. Gregg 111

8 Paroxysmal Nocturnal Hemoglobinuria Charles J. Parker 117

9 Folate, Cobalamin, and Megaloblastic Anemias Ralph Green 133

10 Iron Metabolism Tomas Ganz 169

11 Iron Deficiency and Overload Tomas Ganz 181

12 Anemia Resulting from other Nutritional Deficiencies Ralph Green Ananya Datta Mitra 205

13 Anemia Associated with Marrow Infiltration Vishnu V.B. Reddy Diana Morlote 213

Part IV

Anemias Resulting Principally from Inherited Disorders

14 The Hereditary Dyserythropoietic Anemias Achille Iolascon Roberta Russo Rami Khoriaty 219

15 Erythrocyte Membrane Disorders Theresa L. Coetzer 229

16 Erythrocyte Enzyme Disorders Marije Bartels Eduard J. van Beers Richard van Wijk 259

17 Thalassemia: A Disorder of Globin Synthesis Sujit Sheth Swee Lay Thein 295

18 Disorders of Hemoglobin Structure: Sickle Cell Anemia and other Hemoglobinopathies Vivien A. Sheehan Victor R. Gordeuk Abdullah Kutlar 335

19 Methemoglobinemia and other Dyshemoglobinemias Josef T. Prchal 371

20 Polyclonal and Hereditary Sideroblastic Anemias Amel Hamdi Josef T. Prchal 385

21 The Porphyrias John D. Phillips Karl E. Anderson 395

Part V

Anemia as a Result of Exogenous Factors

22 Fragmentation Hemolytic Anemia Kelty R. Baker Joel Moake 423

23 Erythrocyte Disorders as a Result of Toxic Agents Paul C. Herrmann 431

24 Hemolytic Anemia Resulting from Infections with Microorganisms Marshall A. Lichtman 437

25 Hypersplenism and Hyposplenism Srikanth Nagalla Jaime Caro 447

26 Hemolytic Anemia Resulting from Immune Injury Charles H. Packman 457

27 Alloimmune Hemolytic Disease of the Fetus and Newborn Ross M. Fasano Jeanne E. Hendrickson Naomi L.C. Luban 481

Part VI

Polyclonal Erythrocytosis

28 Primary and Secondary Erythrocytoses (Polycythemias) Josef T. Prchal 499

Part VII

Red Cell Transfusion

29 Erythrocyte Antigens and Antibodies Christine Lomas-Francis 521

30 Blood Procurement and Red Cell Transfusion Claudia S. Cohn Jeffrey McCullough Xiangrong He 545

Index 565

Editorial Reviews

Reviewer: George M Rodgers, MD, PhD (University of Utah School of Medicine)
Description: This book represents a "carve out" of the recently published 10th edition of Williams Hematology (McGraw Hill, 2021) regarding red blood cells and their disorders. Thirty chapters from the main textbook related to red cell physiology and biochemistry, erythropoiesis, anemias, erythrocytosis, etc. are included in this book.
Purpose: The book's purpose is to provide an inclusive text on red cells and their disorders. While this material is present in comprehensive hematology textbooks such as Williams Hematology, 10th Edition or Wintrobe's Clinical Hematology, 14th Edition (Wolters Kluwer, 2019), readers focusing on red cells and their disorders may prefer this targeted text.
Audience: Most students, trainees, and practitioners will prefer a comprehensive textbook since their learning needs and practices usually cover multiple aspects of hematology. Academic hematologists whose efforts are devoted to red cell disorders may prefer this focused text. Both basic scientists and clinical hematologists should find this book to be a useful resource. The editors are noted hematologists in red blood cells and their disorders.
Features: This book is essentially an extraction of 30 red blood cell chapters from the Williams Hematology, 10th Edition textbook, which have been collated into a separate book with a red blood cell focus. The editors and chapter authors of this book are the same as the main textbook. Several chapters have additional references compared to the textbook, indicating that new material is present in the red cell book.
Assessment: This is a unique book. For scientists and clinicians who prefer a focused book on this subject, it is an attractive product and less expensive than Williams Hematology. However, the comprehensive textbook offers a detailed review of all hematology topics. The red cell book offers an updated presentation of the textbook chapters.

Doody's Review Service