Additional Information
Book Details
Abstract
This issue of Hematology/Oncology Clinics, guest edited by Drs. Matthew Heeney and Alan Cohen, is devoted to Iron Disorders. Articles in this issue include: Hereditary Hemochromatosis (HFE and Non-HFE); Iron Refractory Iron Deficiency Anemia (IRIDA); Sideroblastic Anemia; Anemia of Chronic Disease/Inflammation; Pathophysiology of Transfusional Iron Overload; Transfusional Iron Overload and Iron Chelation Therapy; Iron Overload and its Management in Non-Transfusion-Dependent Thalassemia; Treatment of Iron Deficiency Anemia; and Iron Overload Assessment.
Table of Contents
| Section Title | Page | Action | Price |
|---|---|---|---|
| Front Cover | Cover | ||
| Iron Disorders\r | i | ||
| Copyright\r | ii | ||
| Contributors\r | iii | ||
| Contents\r | v | ||
| Hematology/Oncology Clinics Of North America\r | viii | ||
| Preface\r | ix | ||
| Diagnostic Evaluation of Hereditary Hemochromatosis (HFE and Non-HFE) | 625 | ||
| Key points | 625 | ||
| Introduction | 625 | ||
| Iron Metabolism | 625 | ||
| Iron absorption and export | 625 | ||
| Hepcidin | 626 | ||
| Hepcidin regulation | 626 | ||
| Iron Overload | 626 | ||
| Hepcidin deficiency | 626 | ||
| Ferroportin disease | 626 | ||
| Penetrance | 627 | ||
| Diagnostic Work-up | 627 | ||
| Patient history | 627 | ||
| Physical examination | 628 | ||
| Organs Involved in Hereditary Hemochromatosis | 628 | ||
| Liver disease | 628 | ||
| Bones and joints | 628 | ||
| Heart | 629 | ||
| Pancreas | 629 | ||
| Pituitary | 629 | ||
| Clinical Presentation According the Type of Hereditary Hemochromatosis | 630 | ||
| Hepcidin deficiency | 630 | ||
| Ferroportin disease | 630 | ||
| Types of hereditary hemochromatosis and genetic testing | 630 | ||
| The Different Types of Hereditary Hemochromatosis | 630 | ||
| Type 1 (HFE related) hemochromatosis | 630 | ||
| Type 2 (HJV and HAMP related) hemochromatosis | 631 | ||
| Type 3 (TFR2 related) hemochromatosis | 631 | ||
| Type 4 hemochromatosis: ferroportin disease | 631 | ||
| Other rare iron overload diseases | 632 | ||
| Genetic Testing | 632 | ||
| Increased transferrin saturation | 632 | ||
| Normal or low transferrin saturation | 633 | ||
| Summary | 633 | ||
| References | 633 | ||
| Iron-Refractory Iron Deficiency Anemia (IRIDA) | 637 | ||
| Key points | 637 | ||
| Introduction | 637 | ||
| Clinical presentation | 638 | ||
| Genetics | 641 | ||
| Differential diagnosis | 645 | ||
| Treatment | 648 | ||
| Acknowledgments | 648 | ||
| References | 648 | ||
| Sideroblastic Anemia | 653 | ||
| Key points | 653 | ||
| General overview | 653 | ||
| Diagnosis | 654 | ||
| Nonsyndromic CSAs | 654 | ||
| Syndromic CSAs | 658 | ||
| Undefined CSAs | 659 | ||
| Acquired Clonal SAs | 660 | ||
| Acquired Metabolic SAs | 661 | ||
| Disease courses and treatment options | 661 | ||
| Nonsyndromic CSA | 661 | ||
| Syndromic CSA | 662 | ||
| Acquired Clonal SA | 662 | ||
| Splenectomy in SA | 662 | ||
| Iron overload | 663 | ||
| Summary | 665 | ||
| References | 665 | ||
| Anemia of Inflammation | 671 | ||
| Key points | 671 | ||
| Clinical presentation | 671 | ||
| Definitions | 671 | ||
| Related Conditions | 672 | ||
| Diagnosis | 672 | ||
| Diagnostic Challenges | 672 | ||
| Prevalence | 673 | ||
| Pathophysiology | 673 | ||
| Overview of the Causative Factors | 673 | ||
| Erythrocyte Destruction | 673 | ||
| Hypoferremia | 673 | ||
| Increased Hepcidin Causes an Iron-Restricted Anemia Even in the Absence of Inflammation | 674 | ||
| Suppression of Erythropoiesis by Inflammation | 675 | ||
| Hepcidin-induced Hypoferremia and Interferon-γ Synergize to Suppress Erythropoiesis | 675 | ||
| Animal Models of AI Show Partial Dependence on Hepcidin | 676 | ||
| Treatment of AI | 676 | ||
| Current Therapy | 676 | ||
| Experimental Therapy | 678 | ||
| References | 678 | ||
| The Pathophysiology of Transfusional Iron Overload | 683 | ||
| Key points | 683 | ||
| Iron homeostatic mechanisms | 683 | ||
| Body Iron Homeostasis | 684 | ||
| Cellular Iron Homeostasis | 685 | ||
| Impact of blood transfusion on iron balance | 685 | ||
| Thalassemia Major | 686 | ||
| Sickle Cell Disease | 686 | ||
| Other Conditions | 686 | ||
| Mechanisms of iron toxicity in transfusional overload | 687 | ||
| Distribution and consequences of TIO | 688 | ||
| Iron Distribution and Consequences in Thalassemia Major | 688 | ||
| Iron Distribution and Consequences in SCD | 690 | ||
| Iron Distribution in Other Forms of TIO | 691 | ||
| Mechanisms underlying distribution of transfused iron | 691 | ||
| References | 694 | ||
| Transfusional Iron Overload and Iron Chelation Therapy in Thalassemia Major and Sickle Cell Disease | 703 | ||
| Key points | 703 | ||
| Introduction | 703 | ||
| Blood transfusions | 704 | ||
| TM | 704 | ||
| SCD | 704 | ||
| Erythrocytapheresis in SCD | 704 | ||
| Iron overload | 705 | ||
| Does Iron Overload Differ Between SCD and TM? | 705 | ||
| Iron-induced Organ Damage in SCD | 706 | ||
| Complications of Iron Overload | 706 | ||
| Heart | 706 | ||
| Liver | 707 | ||
| Endocrine organs | 707 | ||
| Iron chelation | 708 | ||
| Deferoxamine B | 708 | ||
| Deferiprone | 708 | ||
| Deferasirox | 709 | ||
| New Chelators | 713 | ||
| Combination Therapy | 713 | ||
| New Combinations | 715 | ||
| Alternating Therapy | 715 | ||
| Can Ferritin Be Too Low? | 716 | ||
| Compliance | 716 | ||
| Chelation After Bone Marrow Transplantation | 717 | ||
| Costs | 717 | ||
| Availability of Chelation | 717 | ||
| Summary | 718 | ||
| Supplementary data | 718 | ||
| References | 718 | ||
| Diagnosis and Management of Iron Deficiency Anemia | 729 | ||
| Key points | 729 | ||
| History of iron therapy | 729 | ||
| Problem of IDA | 730 | ||
| Incidence/Prevalence | 730 | ||
| Clinical features and sequelae of IDA | 730 | ||
| Prevention and early diagnosis are the ideal | 731 | ||
| Management of IDA | 732 | ||
| Confirmation of the Diagnosis | 732 | ||
| Other Causes of Microcytic Anemia | 733 | ||
| Identification and Management of the Primary Cause: Children and Adolescents | 733 | ||
| Identification and Management of Primary Cause: Adults | 734 | ||
| Providing Iron Therapy | 734 | ||
| Reasons for Near Absence of Data to Inform Treatment | 734 | ||
| Oral iron therapy | 734 | ||
| Iron Preparation | 734 | ||
| Dosing | 735 | ||
| Hematologic Response and Duration of Therapy | 736 | ||
| Advantages and Disadvantages of Oral Iron | 737 | ||
| Intravenous iron therapy | 737 | ||
| Indications for Intravenous Iron | 737 | ||
| Intravenous Iron Preparations | 737 | ||
| Dosing and Administration | 739 | ||
| Intravenous Iron in Children | 739 | ||
| Oral Versus Intravenous Iron | 739 | ||
| Future research | 740 | ||
| Need for Pragmatic Therapeutic Trials | 740 | ||
| Summary | 740 | ||
| References | 741 | ||
| Use of Magnetic Resonance Imaging to Monitor Iron Overload | 747 | ||
| Key points | 747 | ||
| Monitoring transfusion burden | 747 | ||
| Serum markers of iron overload | 748 | ||
| Measurement of liver iron concentration | 749 | ||
| Liver Biopsy | 749 | ||
| Computed Tomography | 750 | ||
| Magnetic Detectors | 750 | ||
| Magnetic Resonance Imaging | 751 | ||
| Measurement of nonhepatic iron stores | 753 | ||
| Heart Iron | 754 | ||
| Pancreas Iron | 754 | ||
| Pituitary Iron | 757 | ||
| Kidney Iron | 758 | ||
| Spleen Iron | 758 | ||
| Impact of disease state on extrahepatic iron loading | 758 | ||
| Rational monitoring practices | 759 | ||
| Availability of MRI iron assessments | 760 | ||
| Summary | 760 | ||
| References | 761 | ||
| Index | 765 |