Pathophysiology of Blood Disorders / Edition 1

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Overview

A concise full-color review of the mechanisms of blood diseases and disorders – based on a Harvard Medical School hematology course

4 STAR DOODY'S REVIEW!

"This is a superb book. Deceptively small, yet packs a wallop. The emphasis on principles instead of practice is welcome....The text is clear, concise, and surprisingly approachable for what could have been a very dense and dry discussion. I could not put this book down and read it entirely in one sitting. When was the last time anyone found a hematology textbook so riveting?"—Doody's Review Service

Hematological Pathophysiology is a well-illustrated, easy-to-absorb introduction to the physiological principles underlying the regulation and function of blood cells and hemostasis, as well as the pathophysiologic mechanisms responsible for the development of blood disorders. Featuring a strong emphasis on key principles, the book covers diagnosis and management primarily within a framework of pathogenesis.

Authored by world-renowned clinician/educators at Harvard Medical School, Hematological Pathophysiology features content and organization based on a hematology course offered to second year students at that school. The book is logically divided into four sections: Anemias and Disorders of the Red Blood Cell, Disorders of Hemostasis and Thrombosis, Disorders of Leukocytes, and Transfusion Medicine; it opens with an important overview of blood and hematopoietic tissues.

Features

  • Succinct, to-the-point coverage that reflects current medical education
  • More than 200 full-color photographs and renderings of disease mechanisms and blood diseases
  • Each chapter includes learning objectives and self-assessment questions
  • Numerous tables and diagrams encapsulate important information
  • Incorporates the feedback of 180 Harvard medical students who reviewed the first draft — so you know you’re studying the most relevant material possible
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Editorial Reviews

Doody's Review Service
Reviewer: Valerie L. Ng, PhD MD(Alameda County Medical Center/Highland Hospital)
Description: This relatively small paperback book (7 x 9 x 1 inches) contains a wealth of clinical hematology.
Purpose: The book is intended to introduce medical students to the principles and physiology of blood cells, hemostasis, and pathophysiology of blood disorders. These worthy objectives are spectacularly met by the authors.
Audience: The book was modeled on a three-week course given to second-year Harvard medical students, and thus was originally intended for medical students. Physicians at any level of training or practice (except hematology or hematology/oncology specialists who would find this too elementary) would find the book very useful for practical learning or review. Allied health students or practitioners would find it useful as a concise review of hematology. The authors are internationally recognized for their expertise in clinical hematology.
Features: Wow — what can I say? This is a superb book. Deceptively small, yet packs a wallop. The emphasis on principles instead of practice is welcome. Practitioners in the field would do well to use this book as a refresher course for substantive updates since their medical school days. This particular field of medicine tends to superb color displays, well represented in this book. The color figures are simple in design yet rich in content. The photomicrographs, albeit some with imperfect color compensation, are exemplary of the disorders under discussion. The text is clear, concise, and surprisingly approachable for what could have been a very dense and dry discussion. I could not put this book down and read it entirely in one sitting. When was the last time anyone found a hematology textbook so riveting? I was especially grateful to see the emphasis on "benign" hematology — disorders encountered in everyday patient care now being treated by general internists or family practitioners and thus worthy of this attention. The only unusual aspect: the printed text on the title page, preface, table of contents, and index is right up to the edge of the page. The razor-thin margin on these pages does not detract from the intellectual content; it is just jarring to the eye given the beauty evidenced in the rest of the book.
Assessment: Get it if you need a review or want a good read/learning experience in basic clinical hematology. It is a nice companion to Beck's Diagnostic Hematology (Springer, 2009) and Armitage's Atlas of Clinical Hematology, 2nd edition (Springer, 2008).
From The Critics
Reviewer: Valerie L. Ng, PhD MD(Alameda County Medical Center/Highland Hospital)
Description: This relatively small paperback book (7 x 9 x 1 inches) contains a wealth of clinical hematology.
Purpose: The book is intended to introduce medical students to the principles and physiology of blood cells, hemostasis, and pathophysiology of blood disorders. These worthy objectives are spectacularly met by the authors.
Audience: The book was modeled on a three-week course given to second-year Harvard medical students, and thus was originally intended for medical students. Physicians at any level of training or practice (except hematology or hematology/oncology specialists who would find this too elementary) would find the book very useful for practical learning or review. Allied health students or practitioners would find it useful as a concise review of hematology. The authors are internationally recognized for their expertise in clinical hematology.
Features: Wow — what can I say? This is a superb book. Deceptively small, yet packs a wallop. The emphasis on principles instead of practice is welcome. Practitioners in the field would do well to use this book as a refresher course for substantive updates since their medical school days. This particular field of medicine tends to superb color displays, well represented in this book. The color figures are simple in design yet rich in content. The photomicrographs, albeit some with imperfect color compensation, are exemplary of the disorders under discussion. The text is clear, concise, and surprisingly approachable for what could have been a very dense and dry discussion. I could not put this book down and read it entirely in one sitting. When was the last time anyone found a hematology textbook so riveting? I was especially grateful to see the emphasis on "benign" hematology — disorders encountered in everyday patient care now being treated by general internists or family practitioners and thus worthy of this attention. The only unusual aspect: the printed text on the title page, preface, table of contents, and index is right up to the edge of the page. The razor-thin margin on these pages does not detract from the intellectual content; it is just jarring to the eye given the beauty evidenced in the rest of the book.
Assessment: Get it if you need a review or want a good read/learning experience in basic clinical hematology. It is a nice companion to Beck's Diagnostic Hematology (Springer, 2009) and Armitage's Atlas of Clinical Hematology, 2nd edition (Springer, 2008).
Read More Show Less

Product Details

  • ISBN-13: 9780071713788
  • Publisher: McGraw-Hill Professional Publishing
  • Publication date: 1/14/2011
  • Edition description: New Edition
  • Edition number: 1
  • Pages: 342
  • Sales rank: 488,876
  • Product dimensions: 6.90 (w) x 8.90 (h) x 0.60 (d)

Meet the Author

Author Profiles

Dr. Bunn and Dr. Aster are outstanding educators in blood diseases at Harvard Medical School. Dr Bunn is a former president of the American Society of Hematology, and has a great reputation among leading experts in hematology.

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Table of Contents

Table of Contents







Chapter 1 – Overview of Blood and Hematopoietic Tissues




(Aster and Bunn)





Impact of blood in health and disease





Red blood cell





White blood cells





Platelets





Blood clotting proteins





The bone marrow





The spleen





The thymus





Lymph nodes








Chapter 2 – Hematopoiesis and the Bone Marrow




(Scadden)





Hematopoietic cell diffrerentiation





Myeloid lineage





Erythroid lineage





Megakaryocyte-platelet lineage





Lymphoid lineages – B, T, and NK cells





The biology of the stem cell





Self-renewal





Stem cell ontogeny





Stem cell trafficking





The regulation of blood cell formation





The bone marrow niche and cell-cell interactions





Cytokines in early hematopoietic differentiation





Lineage specific cytokines





Cytokine therapy





Stem cell therapy










Section I – Anemias and Disorders of the Red Blood Cell







Chapter 3 – Overview of the Anemias




(Bunn)





(See full sample chapter)





Definition of anemia





Adaptations to anemia





Alterations in blood flow





Changes in oxygen unloading





Stimulation of erythropoiesis





Signs and symptoms of anemia





Pathophysiology of anemia





Anemia due to blood loss





Anemia due to decreased red cell production





Microcytic





Macrocytic





Normocytic





Anemia due to increased red cell destruction




Chapter 4 - Anemias due to Bone Marrow Failure or Infiltration




(Bunn)





Congenital causes of bone marrow failure





Acquired aplastic anemia and pure red cell aplasia





Myelophthisis





Myelodysplasia





Leukemias





(Myelodysplasia and the leukemias will be covered in detail in Chapters 21 and 22).










Chapter 5 – Iron Homeostasis: Deficiency and Overload




(Heeney)





Normal iron homeostasis





Iron binding proteins: transferrin; ferritin





The iron cycle





Role of hepcidin in iron regulation





Iron utilization in erythropoiesis





Laboratory evaluation of iron status





Serum iron and transferrin saturation





Serum ferritin





Bone marrow and liver iron stores





Serum transferrin receptor





Iron deficiency





Etiology





Clinical features – signs and symptoms





Hematological features





Treatment





Iron overload





Primary – inherited mutations in proteins regulating iron homeostasis





Secondary – transfusional hemosiderosis










Chapter 6 - Megaloblastic Anemias




(Heeney)





Biochemistry of vitamin B12 and folate





Pathophysiology





Megaloblastic marrow and peripheral blood morphology





Vitamin B12 and folate absorption





B12 deficiency





Etiology





Clinical presentation (signs and symptoms)





Laboratory evaluation





Treatment





Folate deficiency





Etiology





Clinical presentation (signs and symptoms)





Laboratory evaluation





Treatment












Chapter 7 - Anemias associated with Chronic Disease




(Heeney and Bunn)





Anemia of chronic inflammation





Infection





Cancer





Connective tissue disorders





Pathophysiology – role of hepcidin





Lab features





Treatment





Anemia of renal insufficiency





Cause





Erythropoietin levels





Treatment with erythropoietin and iron





Anemia of chronic liver disease





Anemia of endocrine hypofunction








Chapter 8 – Thalassemia




(Nathan)





Ontogeny of globin gene expression





Organization of alpha and beta globin genes





Definition and classification of the thalassemias





Mutations responsible for the thalassemias





Beta thalassemia





Beta+ versus beta0





Beta thal major





Cellular pathogenesis





Clinical presentation





Laboratory evaluation





Complications





Treatment





Red cell transfusion





Iron chelation





Stem cell transplant





Prevention – prenatal diagnosis





Beta thal intermedia





Beta thal minor





Interacting beta thalassemias – Hb S and Hb E





Alpha thalassemia





Four degrees of gene deletion – correlate with clinical presentation





Three alpha gene deletion - Hb H disease





Four alpha gene deletion – Hydrops fetalis





Prevention – prenatal diagnosis








Chapter 9 – Sickle Cell Disease and other Disorders of Hemoglobin Structure




(Bunn)





Inheritance – beta globin structural mutation:

b

6 Glu

®

Val





The sickling disorders: SS, S

b


0

Thal, S

b


+

Thal

, SC

, AS





Molecular pathogenesis





Structure of the sickle fiber





Kinetics of fiber formation





In vivo significance of polymer formation





Cellular aspects of in vivo sickling and vaso-occlusion





Contribution of Hb F





Sickle cell – endothelial cell adhesion





Clinical manifestations





Constitutional: growth, development and susceptibility to infections





Hemolytic anemia





Vaso-occlusion





Acute pain crises





Acute chest syndrome





Chronic organ damage





Stroke





Bone – aseptic necrosis





Renal: impaired concentrating ability; impaired glomerular function





Pulmonary hypertension





Treatment





Supportive – analgesia, oxygen, fluid and pH balance





Prophylaxis: penicillin and vaccinations





Hydroxyurea – induction of Hb F





Novel therapeutic strategies
















Chapter 10 – Other Inherited Hemolytic Anemias




(Lux)





Disorders of the red cell membrane





Molecular anatomy of the red cell membrane





Hereditary spherocytosis – mutations in spectrin, band 4.1, band 3





Other inherited membrane disorders





Disorders of red cell metabolism





Hexose monophosphate shunt and G6PD deficiency





Glycolytic pathway – pyruvate kinase deficiency








Chapter 11 – Acquired Hemolytic Anemias




(Bunn)





Acquired membrane disorders





Paroxysmal nocturnal hemoglobinuria





Spur cell anemia





Traumatic hemolytic disorders





Thrombotic thrombocytopenic purpura (Covered in detail in Chapter 14)





Hemolytic uremic syndrome





Disseminated intravascular coagulation (Covered in detail in Chapter 16)





Heart valve hemolysis





Immune hemolytic anemias





Pathophysiologic principles





Clinical presentation and course





Warm antibody hemolysis





Cold antibody hemolysis





Lab diagnosis





Treatment








Chapter 12 - Erythrocytosis (Polycythemia)




(Bunn)





Pathophysiologic principles:

Algorithm for evaluating patients with erythrocytosis





Primary erythrocytosis – polycythemia vera





(See Chapter 20 for coverage of molecular pathogenesis, Chapter 22 for clinical presentation and course, diagnosis and treatment)





Secondary erythrocytosis





Appropriate erythropoietin production





High altitude hypoxemia





Pulmonary hypoxemia





Cardiac hypoxemia (right to left shunt)





Mutant hemoglobin with high oxygen affinity





Inapproriate erythropoietin production





Tumors: renal, hepatic





Von Hippel Lindau syndrome





Inherited disorders of oxygen sensing HIF pathway










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