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| About the Programme |
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The European School of Haematology (ESH) is pleased to present this free online educational tool for haematologists and other clinicians involved in the care of patients with iron metabolism disorders. The high-level comprehensive curriculum features the very latest information about iron metabolism and the management of related disorders, and is designed to help you provide state-of-the-science treatment to improve your patients' outcomes.
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| Co-Chairs: |
Photis Beris, MD
Professor of Clinical
Haematology
Department of Internal
Medicine
Geneva University Hospital
Geneva, Switzerland
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Pierre Brissot, MD
Professor of Medicine
Liver Disease Department
University Hospital
Pontchaillou
Rennes, France
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Maria D. Cappellini, MD
Professor of Internal
Medicine
University of Milan
Maggiore Hospital
Milan, Italy
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| Programme Overview |
Iron Metabolism
- Molecular physiology of iron metabolism
- Factors contributing to iron pathophysiology
- Role of hepcidin, ferroportin, transferrin, NTBI, LPI in iron metabolism
- Brief overview of spectrum of chronic overload diseases
- Transfusional iron overload
- Myelodysplastic syndromes (MDS)
- Sickle cell disease (SCD)
- Thalassaemia
- Other transfusional anaemias
- Genetic iron overload
- Hereditary haemochromatosis (classic)
- Juvenile haemochromatosis
- Brief overview of visceral targets of iron excess
- Heart
- Liver
- Endocrine system (including diabetes)
- Musculoskeletal system
Epidemiology and Disease Pathophysiology
- Myelodysplastic syndromes (MDS)
- MDS epidemiology
- MDS pathophysiology
- MDS diagnosis and management
- Thalassaemia
- Thalassaemia epidemiology
- Thalassaemia pathophysiology
- Thalassaemia diagnosis and management
- Sickle cell disease (SCD)
- SCD epidemiology
- SCD pathophysiology
- SCD diagnosis and management
- Other anaemias
- Epidemiology of other anaemias
- Pathophysiology of other anaemias
- Diagnosis and management of other anaemias
- Hereditary Haemochromatosis
- HH epidemiology
- HH pathophysiology
- HH diagnosis and management
Iron Deficiency
- Epidemiology of iron deficiency
- Pathophysiology of iron deficiency
- Clinical sequelae of iron deficiency
- Diagnosis and management of iron deficiency
Iron Toxicity and Clinical Sequelae
- Molecular basis of iron overload
- Causes of iron overload
- Primary causes of iron overload (hereditary)
- Hereditary haemochromatosis (classic)
- Juvenile haemochromatosis
- African iron overload
- Secondary causes of iron overload (transfusional)
- Myelodysplastic syndromes (MDS)
- Sickle cell disease (SCD)
- Thalassaemia
- Other transfusional anaemias
- Clinical sequelae of iron overload
- Heart
- Microscopic consequences of iron overload in the heart
- Functional consequences of iron overload in the heart
- Liver
- Microscopic consequences of iron overload in the liver
- Functional consequences of iron overload in the liver
- Endocrine system (including diabetes)
- Microscopic consequences of iron overload in the endocrine system
- Functional consequences of iron overload in the endocrine system
- Musculoskeletal system
- Microscopic consequences of iron overload in the musculoskeletal system
- Functional consequences of iron overload in the musculoskeletal system
- Link to cancer
- HCC, esophageal and intestinal cancer, melanoma, and lung cancer
- Others
- Morbidity and mortality associated with iron overload/toxicities
Diagnosis of Iron Overload
- Methodologies for diagnosing and monitoring iron overload
- Established methodologies
- Liver biopsy - invasive, direct measurement
- Review of methodology
- Review of factors affecting the accuracy of liver biopsy measurements
- Serum ferritin - non-invasive, indirect measurement
- Review methodology
- Review of factors affecting the accuracy of serum ferritin measurements
- Investigational methodologies
- Superconducting quantum interference device (SQUID) - non-invasive, indirect measurement (limited availability)
- Review methodology
- Review of factors affecting the accuracy of SQUID measurements
- MRI - non-invasive, indirect measurement
- Review methodology that includes
- R2 relation rate (St Pierre R2) to assess LIC
- R2 relation rate (FAST Wood R2)
- Signal density ratio (SIR) of R2-weighted images (Jensen R2 SIR)
- SIR of R2*-weighted images (Gandon R2* SIR)
- Review of factors affecting the accuracy of MRI measurements
- Others
- Statistical modeling of LIC
- New biomarkers for iron overload
- Monitoring to achieve clinical goals
Iron Chelator Basics (including animation)
- Goals of ideal iron chelator
- Properties
- Prevention or modification of iron-mediated pathological processes
- Prevention
- Rescue
- Detoxification of labile iron - 24-hour coverage
- Wide therapeutic safety margin
- Patient compliance
- Review of efficacy, safety profiles of currently available iron chelators (also in grid format), and patient preferences
- Desferrioxamine
- Characteristics (including half-life) and method of administration
- Efficacy data
- Safety/side effect profile
- Advantages and disadvantages
- Deferiprone
- Characteristics (including half-life) and method of administration
- Efficacy data
- Safety/side effect profile
- Advantages and disadvantages
- Deferasirox
- Characteristics (including half-life) and method of administration
- Efficacy data
- Safety/side effect profile
- Advantages and disadvantages
- Importance of patient compliance for improved outcomes
- Iron Chelator Grid differentiating iron chelators
Indications for Successful Iron Overload Treatment and Monitoring
- Review of differences in approaching disease specific states of iron overload
- Guideline updates
- State-of-the-science disease-specific iron chelation treatment of iron overload
- Myelodysplastic syndromes (MDS)
- Appropriate patient selection in MDS
- Administration in MDS
- Treatment options and rationale based on efficacy/safety considerations in MDS
- Monitoring in MDS
- Sickle cell disease (SCD)
- Appropriate patient selection in SCD
- Administration in SCD
- Treatment options and rationale based on efficacy/safety considerations in SCD
- Monitoring in SCD
- Thalassaemia (major and intermedia)
- Appropriate patient selection in thalasaemia
- Administration in thalasaemia
- Treatment options and rationale based on efficacy/safety considerations in thalasaemia
- Monitoring in thalasaemia
- Other transfusional anaemias
- Appropriate patient selection in other transfusional anaemias
- Administration in other transfusional anaemias
- Treatment options and rationale based on efficacy/safety considerations in other transfusional anaemias
- Monitoring in other transfusional anaemias
- Hereditary haemochromatosis (classic)
- Appropriate patient selection in HH
- Administration in HH
- Treatment options and rationale based on efficacy/safety considerations in HH
- Monitoring in HH
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Iron Metabolism & Related Disorders in the Following Disease States |
- Myelodysplastic Syndromes
- Sickle Cell Disease
- Thalassaemia (major and intermedia)
- Other Transfusion-Dependent Anaemias
- Hereditary Haemochromatosis
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| Covered Topics Include |
- Iron Metabolism
- Epidemiology and Disease Pathophysiology
- Iron Deficiency
- Iron Toxicity and Clinical Sequelae
- Diagnosis of Iron Overload
- Iron Chelator Basics (including animation)
- Indications for Successful Iron Overload Treatment and Monitoring
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