The BSH Guidelines Official Podcast
Guidelines for the monitoring and management of iron overload in patients with haemoglobinopathies and rare anaemias

Guidelines for the monitoring and management of iron overload in patients with haemoglobinopathies and rare anaemias

May 3, 2022

Dr Shivan Pancham and Dr Farrukh (Farrah) Shah both present a podcast on the Guidelines for the monitoring and management of iron overload in patients with haemoglobinopathies and rare anaemias.

 

Both Dr Pancham and Dr Shah discuss the guideline in three main parts:

1) Diagnose and complications of iron overload (Dr Shah)

2) Medications use to treat iron overload (Dr Pancham and Dr Shah)

3) Initialization of iron chelation therapy and maintenance treatment and monitoring for complications of therapy (Dr Pancham and Dr Shah)

Iron overload (IOL), resulting from regular or intermittent blood transfusions or from increasing dietary iron absorption can cause serious and life-threatening complications. Patients at risk of IOL include those with inherited anaemias such as transfusion-dependent thalassaemia (TDT) and non-transfusion-dependent thalassaemia (NTDT), transfused sickle cell disease (SCD) and rarer anaemias such as congenital sideroblastic anaemia (CSA), congenital dyserythropoietic anaemia (CDA), Diamond-Blackfan anaemia (DBA) as well as red cell enzymopathies, membrane disorders and defects in haem synthesis pathways. The United Kingdom has approximately 15 000 patients with these disorders and diagnosis and management of IOL is important in minimising morbidity and mortality. Other disorders that are associated with IOL such as hereditary haemochromatosis or acquired anaemias such as the myelodysplastic syndromes are not covered by this guideline.

The extent and severity of IOL is affected by both the underlying disorder and the intensity and duration of transfusion. Patients on regular top-up transfusions are at most risk whilst those on intermittent transfusions develop IOL more slowly. In the absence of blood transfusion, sickle cell disorders tend not to accumulate excess iron: however, manual and automated exchange transfusion may result in mild degrees of IOL or even iron deficiency.1-3

Dr Shivan Pancham is a  Consultant Haematologist with Sandwell and West Birmingham NHS Trust. She is one of two lead clinicians for the West Midlands Sickle HCC. She has been a writing group member for the national sickle and thalassaemia guidelines.

Dr Farrukh (Farrah) Shah is a Consultant Haematologist at both the Whittington hospital in London and NHS Blood and Transplant (Colindale). Dr Shah has a specialist interest in the management of transfusional iron overload and haemoglobinopathies. She is actively involved in clinical research in iron chelation and is a local investigator and national investigator for a number of clinical trials.

 

Good Practice Paper for the laboratory diagnosis of iron deficiency in adults (excluding pregnancy) and children

Good Practice Paper for the laboratory diagnosis of iron deficiency in adults (excluding pregnancy) and children

April 22, 2022

Dr Andrew Fletcher presents a podcast on the Good Practice Paper for the laboratory diagnosis of iron deficiency in adults (excluding pregnancy) and children.

Dr Fletcher discusses the guideline in three main parts:

1) Laboratory investigations and testing for iron deficiency

2) Advantages and disadvantages of measuring blood counts and ferritin

3) Recommendations

The laboratory diagnosis of iron deficiency is difficult because iron homeostasis is dynamic. No single test can provide an accurate assessment of iron absorption, transport, storage, and utilisation. The different assays available to assess iron and its stores will each be discussed, and recommendations made pertinent to practice within the UK. Iron metabolism in adults and children can be considered equivalent and these recommendations are applicable to both paediatric and adult practice.

 

Dr Andrew Fletcher is a Consultant Haematologist in paediatric haematology at the Great North Children's Hospital The Newcastle upon Tyne Hospitals NHS Foundation Trusts. His specialist interests are in laboratory medicine, non-malignant haematology and acute myeloid leukaemia.

Guidelines on the diagnosis and management of Waldenström macroglobulinaemia

Guidelines on the diagnosis and management of Waldenström macroglobulinaemia

January 20, 2022

Dr Dima El-Sharkawi presents a podcast on the Guidelines on diagnosis and management of Waldenström macroglobulinaemia

Dr El-Sharkawi discusses the guideline in three main parts:

1) Presentation of diagnosis of Waldenström macroglobulinaemia

2) Treatment of Waldenström macroglobulinaemia

3) Common complications of Waldenström macroglobulinaemia

Dr Dima El-Sharkawi is a Consultant Haematologist and part of the haematological malignancy diagnostic services at the Royal Marsden NHS Foundation Trust. She is also a Trustee for the UK Charity for Waldenström macroglobulinaemia (WMUK). Dr El-Sharkawi's special interests are in CLL, rare leukaemias and lymphomas including Waldenström macroglobulinaemia.

Guidelines for the management of mature T- and natural killer-cell lymphomas (excluding cutaneous T-cell lymphoma)

Guidelines for the management of mature T- and natural killer-cell lymphomas (excluding cutaneous T-cell lymphoma)

December 2, 2021

Dr Christopher Fox presents a podcast on the Guidelines for the management of mature T- and natural killer-cell lymphomas (excluding cutaneous T-cell lymphoma).

Dr Fox discusses the guideline in three main parts:

1) Background and context on this diesease area; reflects on the progress that has been made over the past decade.

2) General recommendations from the guideline.

3) Specific disease areas where some progress has been made in clinical treatments and in other disease areas where more work is needed.

Dr Christopher Fox is a consultant Haematologist at the Nottingham University Hospital and Honourary Associate Professor at the University of Nottingham. He is the Chair of the UK National Cancer Research Institute Aggressive Cancer Study Group (NCRI). Dr Fox's main background and interest are in the field of T-cell Lymphoma including being a member of the international T-cell project.

 

Guideline on The management of Castleman disease

Guideline on The management of Castleman disease

October 27, 2021

Prof Steve Schey presents a podcast on the Guideline on The management of Castleman disease.

 

Prof Schey discusses management and best outcome treatments of Castleman disease.

 

Professor Steve Schey is a Consultant Haematologist and Head of Myeloma Services at Kings College Hospital, London. He is one of the founding members of the Castleman Disease Collaborative Network. He is a trustee of the lymphoma research trust. He is Chairman of the NCRI Myeloma Clinical Trials Committee. He has been Chief Investigator and Co-investigator for a large number of clinical trials. His research interests are in multiple myeloma and the bone marrow microenvironment as potential novel therapeutic targets, and haemopoietic stem cell transplantation

Good Practice Paper Richter transformation of chronic lymphocytic leukaemia

Good Practice Paper Richter transformation of chronic lymphocytic leukaemia

October 25, 2021

Dr Toby Eyre presents a short podcast on the Good Practice Paper Richter transformation of chronic lymphocytic leukaemia

 

Dr Eyre discusses the following: 

1) Diagnostic work up of richter transformation

2) Current treatments available in this disorder

3) Future developments and ongoing trials

 

Dr Toby Eyre is a Consultant Haematologist and honorary senior lecturer at Oxford University Hospitals NHS Foundation Trust, specialising in the management of lymphoid malignancies. He is considered a national expert in the management of patients with lymphoma and patients with chronic lymphocytic leukaemia. He is heavy involved in clinical trials of richter syndrome in the UK. Some of his special interests include acute myeloid leukaemia, chronic myeloid leukaemia, hodgkin lymphoma and myeloma. Dr Eyre holds the position of secretary on the BSH Haematology Oncology Task Force. 

Good Practice Paper: Cytomegalovirus serological testing in potential allogeneic haematopoietic stem cell transplant recipients

Good Practice Paper: Cytomegalovirus serological testing in potential allogeneic haematopoietic stem cell transplant recipients

October 7, 2021

Dr Suzy Morton presents a short podcast on the Good Practice Paper: Cytomegalovirus serological testing in potential allogeneic haematopoietic stem cell transplant recipients. 

Dr Morton discusses the following:

1) The background and importance of cytomegalovirus serological testing

2) Explaining the recommendations

3) A short summary of the importance on key messages from this good practice paper

 

Dr Suzy Morton is a Consultant in clinical haematology and blood transfusion at the Queen Elizabeth Hospital, University Hospitals Birmingham and NHS Blood and Transplant. She is the transfusion representative on the West Midlands Haematology Specialty Training Committee and the educational lead for haematology SpRs at QEHB. Suzy is a transfusion representative on the BSH Education committee.

Good Practice Paper: Management of cardiovascular complications of bruton tyrosine kinase inhibitors (BTKi)

Good Practice Paper: Management of cardiovascular complications of bruton tyrosine kinase inhibitors (BTKi)

September 13, 2021

Dr Nilima Parry Jones introduces the podcast for the Good Practice Paper: Management of cardiovascular complications of bruton tyrosine kinase inhibitors (BTKi).

This good practice paper aims to help clinicians in recognising, understanding and appropriately managing cardiovascular complications of BTKi in order to optimise outcomes for patients.

The podcast is to take the form of a question and answer session between Dr Renata Walewska and Professor Gregory Lip.

 

Dr Nilima Parry-Jones is a Consultant Haematologist at the Aneurin Bevan Local Health, Wales and an executive member of the UK CLL Forum.

Dr Renata Walewska is a Consultant Haematologist at the Royal Bournemouth Hospital and Chair of the UK CLL Forum.

Professor Gregory Lip is Price-Evens Chair of Cardiovascular Medicine at the University of Liverpool and Director of the Liverpool Centre for Cardiovascular Science and the University of Liverpool and Liverpool Heart & Chest Hospital.

Guidelines for the clinical and laboratory diagnosis of heritable platelet disorders in adults and children

Guidelines for the clinical and laboratory diagnosis of heritable platelet disorders in adults and children

August 27, 2021

Dr Keith Gomez presents a short podcast on the Guidelines for the clinical and laboratory diagnosis of heritable platelet disorders in adults and children.

 

Dr Gomez discusses the following:

1) Why we need this guideline now

2) Clinical assessment of heritable platelet disorders

3) Laboratory investigations of heritable platelet disorders

 

This guideline replaces the previous British Committee for Standards in Haematology guideline published in 2011 on laboratory diagnosis of heritable disorders of platelet function.1 The remit has been expanded to include clinical diagnosis and heritable thrombocytopenia under the overarching term heritable platelet disorder (HPD). Acquired disorders such as immune thrombocytopenia and drug-induced platelet dysfunction are not covered.

Also, Dr Gomez presented this guideline at the BSH ASM Guidelines session and this is available to view.

Dr Keith Gomez is the Chair of the BSH Haemostasis and Thrombosis Task Force and the President of the British Society of Haemostasis and Thrombosis. He currently is a Consultant Haematologist and Associate Professor in Haemostasis in the Haemophilia Centre and Thrombosis Unit at the Royal Free Hospital in London. 

 

Guideline on Management of sickle cell disease in pregnancy

Guideline on Management of sickle cell disease in pregnancy

August 20, 2021

Dr Eugene Oteng-Ntim presents a short podcast on the Guideline on Management of sickle cell disease in pregnancy.

The purpose of this guideline is to describe the management of sickle cell disease (SCD) in pregnancy in the UK.

It will cover the following: preconception screening and antenatal

Intrapartum and postnatal management of women with the condition.

It will not cover the management of women with sickle cell trait. Updates from the previous guideline1 include new information on pre-implantation genetic diagnosis (PGD), more comprehensive information on pre-conceptual screening and medication review, updated information on thromboprophylaxis, aspirin and vitamin D, changes to advice on antenatal care including frequency of ultrasonography (USS) scanning. It also includes reference to the most recent National Institute for Health and Clinical Excellence (NICE) and RCOG guidelines. 

Also, this guideline was presented at the Guidelines Session at the ASM 2021 and the presentation can be found in our list of podcasts.

Dr Oteng-Ntim is a Consultant Obstetrician and Head of Obstetrics at Guy's and St Thomas NHS Trust Foundation. He is a Honorary Reader in Women's Health (KCL) and Honorary Associate Professor in Epidemiology and Population Health (LSHTM).

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