Short podcasts on current haematological guidelines and good practices papers produced by the British Society for Haematology
British Society for Haematology
Guideline on the Investigation and management of a raised serum ferritin: A podcast recording of a conversation between Dr Jenny Tam and Dr Wayne Thomas.
Antenatal screening/testing of pregnant women should be carried out according to the guidelines of the National Health Service (NHS) Sickle Cell and Thalassaemia Screening Programme. Newborn screening and, when necessary, follow-up testing and referral, should be carried out according to the guidelines of the NHS Sickle Cell and Thalassaemia Screening Programme. All babies under 1 year of age arriving in the United Kingdom should be offered screening for sickle cell disease (SCD). Preoperative screening for SCD should be carried out in patients from ethnic groups in which there is a significant prevalence of the condition. Emergency screening with a sickle solubility test must always be followed by definitive analysis. Laboratories performing antenatal screening should utilise methods that are capable of detecting significant variants and are capable of quantitating haemoglobins A2 and F at the cut-off points required by the national antenatal screening programme. The laboratory must ensure a provisional report is available for antenatal patients within three working days from sample receipt.
The objective of this guideline is to provide healthcare professionals with clear guidance on the diagnosis and management of patients with mantle cell lymphoma.
This Good Practice Paper podcast provides recommendations for the diagnosis, risk stratification and management of the monoclonal gammopathy of undetermined significance (MGUS). It describes the recently recognised entity of the monoclonal gammopathy of clinical significance (MGCS), and recommends how it should be managed. The potential for targeted population screening for MGUS is also discussed.
Haematological management of major haemorrhage: A podcast recording of a conversation between Prof Simon Stanworth and Dr Heidi Doughty.
A BSH Guideline on the Anticoagulant management of pregnant individuals with mechanical heart valves presented by Dr Will Lester.
Guideline session at EHA Conference 2022 on The Use of Next-generation Sequencing in the Diagnosis of Rare Inherited Anaemias: A Joint BSH/EHA Good Practice Paper
Secondary central nervous system (CNS) lymphoma (SCNSL) refers to lymphoma that has spread to the CNS concurrently with, or following treatment for, systemic lymphoma. There are three clinically distinct scenarios: Synchronous CNS and systemic lymphoma at initial presentation (treatment-naïve; TN-SCNSL), CNS relapse without recurrent systemic lymphoma (relapsed isolated CNS lymphoma; RI-SCNSL). Relapsed concomitant systemic and CNS disease following treatment for systemic lymphoma (RC-SCNSL). CNS lymphoma (CNSL) is associated with inferior outcomes, which may be attributed to several factors: poor CNS penetrance of chemotherapeutics, including RCHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone),1 impaired neurocognitive function and patient performance status (PS) contributing to increased treatment toxicity,2, 3 and recurrent genetic aberrations conferring treatment resistance.4-6 The rarity and heterogeneity of SCNSL also limits the evidence base for treatment recommendations, with poor outcomes potentially attributable at least in part to lack of optimised treatment protocols. This good practice paper focuses on diffuse large B-cell lymphoma (DLBCL), the most common SCNSL subtype. It covers diagnostic and therapeutic aspects of care for the three SCNSL scenarios and multiply relapsed SCNSL. Treatment recommendations are framed by patient fitness and treatment intent.
This guideline updates and widens the scope of the previous British Society for Haematology (BSH) Clinical guidelines for testing for heritable thrombophilia1 to include both heritable and acquired thrombophilia. The term thrombophilia is generally used to describe hereditary and/or acquired conditions associated with an increased predisposition to thrombosis. Heritable thrombophilia refers to genetic disorders of specific haemostatic proteins. These guidelines focus only on the factors that are identified from laboratory testing and therefore exclude disorders such as cancer, inflammatory conditions and obesity that are associated with thrombosis through multiple mechanisms.
Audio commentary from Dr Mike Dennis on the good practice paper - management of older patients with frailty and acute myeloid leukaemia.
Audio commentary by Dr George Follows.
British Society for Haematology Annual Scientific Meeting Guidelines Session 2022 Dr Suzy Morton on the Good Practice Paper Cytomegalovirus serological testing in potential allogeneic haematopoietic stem cell transplant recipients. 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.
British Society for Haematology Annual Scientific Meeting Guidelines Session 2022 Dr Katie Hands presents on the Good Practice Paper Preoperative patient blood management during the SARS-CoV-2 pandemic. Katie Hands is a Consultant Haematologist with the Scottish National Blood Transfusion Service (SNBTS) based at Ninewells Hospital, Dundee. She is a member of the British Society for Haematology Transfusion Task Force and is involved in the preparation of evidence-based guidelines relating to all aspects of blood transfusion in the United Kingdom.
British Society for Haematology Annual Scientific Meeting Guidelines Session 2022 Dr Austin Kulasekararaj presents on the Myelodysplastic syndromes covering the BSH Guidelines on the diagnosis and prognosis of adult myelodysplastic syndromes and Guidelines on the management of adult myelodysplastic syndromes. Dr Austin Kulasekararaj is a Consultant Haematologist at the King's College Hospital London. His special interests are adult myelodysplastic syndromes, acute leukaemia and aplastic anaemia.
British Society for Haematology Annual Scientific Meeting Guidelines Session 2022 Dr Dima ElSharkawi presents the Guideline on the Diagnosis and Management of Waldenstrom 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.
Dr Nilima Parry Jones and Dr Renata Walewska present a podcast on the Guideline for the treatment of chronic lymphocytic leukaemia Both Dr Parry Jones and Dr Walewska discuss the guideline in three main parts: 1) Choice of treatment for CLL in front line and relapsed settings 2) Impact to immune dysregulation in respect of anti-microbial prophylaxis and vaccination 3) Challenges of the Covid pandemic, the evolving evidence on vaccine response, treatment of patients with Covid and antiviral and monoclonal antibodies Dr Nilima Parry-Jones is a Consultant Haematologist at the Aneurin Bevan Local Health, Wales and an executive member of the UK CLL Forum. She is the Chair of the BSH Haematology Oncology Task Force. Dr Renata Walewska is a Consultant Haematologist at the Royal Bournemouth Hospital and Chair of the UK CLL Forum.
Dr Noemi Roy and Dr Roberta Russo present a podcast on the The use of next-generation sequencing in the diagnosis of rare inherited anaemias: A Joint BSH/EHA Good Practice Paper Dr Roy and Dr Russo discuss the good practice paper in three main parts: 1) Focus on rare anaemias: ( Diamond-Blackfan Anaemia, Congenial dyserythropoietic anaemia (CDA), Sideroblastic anaemia and Red cell membrane/cation leaking and enzyme disorders 2) Next generation sequencing (NGS) 3) How and why we need to interpret genetic variants carefully The use of next-generation sequencing (NGS) in the diagnosis of rare inherited anaemias is increasingly common, as evidenced by a growing number of publications describing its clinical utility.1-6 Excluding disorders of globin synthesis, rare anaemias include Diamond–Blackfan anaemia (DBA), congenital dyserythropoietic anaemias (CDA), congenital sideroblastic anaemias (CSA) and disorders of red cell membrane and enzymes. Other forms of genetic anaemias can also be considered while establishing NGS panels, in particular genetic syndromes where anaemia comprises one of the constellation of symptoms. Dr Noemi Roy is a Consultant Haematologist at Oxford University Hospitals NSH Foundation Trust and honorary senior clinical lecturer in haematology at the University of Oxford. She is a BSH General Haematology Task Force member and Chair for the Guidelines Executive committee for the European Hematology Association. She is the rare anaemia representative at the National Haemoglobinopathy Panel. Her main interests are haemoglobinopathies and rare inherited anaemias, and in particular the genetic investigation of inherited anaemias, including whole genome sequencing. Dr Roberta Russo is an assistant professor in medical genetics at the University of Naples "Federico II". She has dealt with the study of the genetics of rare disorders, particularly hereditary anaemias, since 2008. Her main interest was the study of Congenital Dyserythropoietic Anaemias (CDAs), mainly CDA type II, genetics and genomics of red cell anaemias and blood cell defects . She contributed first hand to the identification of the causative genes of rare anaemias. Subsequently, she became one of the leading scientists for the genetics and epidemiology of CDAs. Since 2015, she has been dealing with the application of next generation sequencing to the clinical and medical definition of patients affected by this conditions. In 2011, she won the Young Investigator Award by the American Journal of Hematology for her study on the epidemiology of this disorder.
Professor Peter Chiodini present a podcast on the Guidelines for the laboratory diagnosis of malaria. Professor Chiodini will discuss the guideline by highlighting points which will be relevant to your practice, giving pointers to where one may need to seek more information and how one may be able to access help in one would like to increase expertise in this critical area. Professor Chiodini begins with explaining why do we need this guideline with the basics of the malaria problem and why we need to continue excellence in diagnosis. Highlights include: 1) Malaria microscopy 2) Diagnosis of malaria by rapid diagnostic tests 3) Malaria molecular methods The range of supplementary tests available for diagnosing malaria has continued to expand. Despite this, carefully examined thick and thin blood films remain an essential part of the process. This guideline updates the previous 2013 British Society for Haematology Guideline for the Laboratory Diagnosis of Malaria. Professor Peter Chiodini is a Consultant Parasitologist at the Hospital for Tropical Diseases, Honorary Professor at the London School of Hygiene & Tropical Medicine, Honorary Clinical Professor at University College London and Director of the Public Health England (PHE) Malaria Reference Laboratory and the PHE Parasitology Reference Laboratory. He organizes the UK National External Quality Assessment Schemes for Parasitology, is a member of the PHE Advisory Committee on Malaria Prevention in Travellers, the Department of Health Advisory Committee on Dangerous Pathogens, and advises the National Blood Service on the prevention of transfusion-transmitted parasitic infections. His research interests include new diagnostic methods for parasitic infections, malaria, Chagas disease and hydatid disease. In 2018 he was awarded an OBE for services to parasitology and malaria.
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.
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.
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.
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.
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
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.
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.
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.
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.
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).
Dr Sally Killick presents a short podcast to discuss prognosis, diagnosis and management of adult MDS on the following guidelines: Guidelines for the diagnosis and evaluation of prognosis of adult myelodysplastic syndromes and Guidelines for the management of adult myelodysplastic syndromes. Dr Sally Killick is a Consultant Haematologist at the University Hospitals Dorset NHS Foundation Trust. She is the chair of the Myelodysplastic syndrome (DS) clinical study group for the National Cancer Research Institute (NCRI). Her area of speciality are myeloid cancers (myelodysplastic syndromes- MDS and myeloproliferative neoplasms) and lymphoma.
British Society Haematology Virtual Annual Scientific Meeting Guidelines Session 2021 General Haematology Presentations Question and Answer with Eugene Oteng-Ntim and Nick Cross; hosted by Jo Howard.
British Society for Haematology Virtual Annual Scientific Meeting Guidelines Session 2021 Professor Nick Cross presents the Good Practice Paper The use genetic tests to diagnose and manage patients with myeloproliferative and myeloproliferative/myelodysplastic neoplasms, and related disorders. The good practice paper aims to be published summer 2021. Professor Nick Cross is Professor of Human Genetics within Medicine at the University of Southampton and Director for Wessex Regional Genetics Laboratory Salisbury.
British Society for Haematology Virtual Annual Scientific Meeting Guidelines Session 2021 Dr Eugene Oteng-Ntim presents on the draft Guidelines Management of sickle cell disease in pregnancy. The guideline is now published . 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).
British Society Haematology Virtual Annual Scientific Meeting Guidelines Session 2021 Haemostasis and Thrombosis Presentations Question and Answer with Keith Gomez and Will Lester; hosted by Jo Howard.
British Society for Haematology Virtual Annual Scientific Meeting Guidelines Session 2021 Dr Will Lester presents on the draft Guidelines on The Assessment of bleeding risk prior to surgery or invasive procedures. The guidelines aims to be published by the end of 2021 early 2022. Dr Lester is a Honorary Haematology Consultant at University Hospitals Birmingham NHS Foundation Trust and deputy chair of the BSH Haemostasis and Thrombosis Task Force.
British Society for Haematology Virtual Annual Scientific Meeting Guidelines Session 2021 Dr Keith Gomez presents on the final draft Guidelines for the laboratory investigation of heritable disorders of platelets function. The guideline aims to be published in the next two to three months. Dr Gomez is an Associate Professor in Haematology and Consultant Haematologist at the Royal Free London NHS Foundation Trust and chair of the BSH Haemostasis and Thrombosis Task Force. He has extensive clinical experience in the management of bleeding disorders, such as haemophilia, and disorders of blood clotting (thrombosis).
Dr Alwyn Kotze presents a short podcast on the BSH Good Practice Paper Preoperative patient blood management during the SARS-CoV-2 pandemic The focus of this good practice paper is to highlight the challenges in provision of elective surgical services while SARS-CoV-2 remains prevalent in communities. Dr Kotze discusses the good practice paper in three sections: 1) How the good practice paper is produced and which evidence is taken in account doing so 2) How we think pandemic times have changed our pathways especially concentrating on remote working and how we believe teams can and should prioritise different aspects inherent in a patient blood management programme. 3) Clinical subject matter pandemic specific on the following: management of preoperative anaemia bleeding risk assessment and investigation medication management of trying to reduce bleeding Dr Alwyn Kotze is a Consultant Anaesthetist at Leeds Teaching Hospitals NHS Trust. Dr Kotze's clinical practices is a mixture of organ transplantation specifically liver transplants and providing other anaesthesia for surgeries.
Dr Jonathan Sive presents a podcast on the BSH Guidelines on the diagnosis, investigation and initial treatment of myeloma. The objective of this guideline is to provide healthcare professionals with clear guidance on the anti‐myeloma management of patients with newly diagnosed multiple myeloma. In all cases, individual patient circumstances may dictate an alternative approach. Dr Sive discusses the guidelines three main focus areas: 1) Initial investigation diagnosis of myeloma 2) Choice of treatment for myeloma 3) Post treatment consolidation therapy Dr Jonathan Sive is a Consultant Haematologist and the clinical service lead for myeloma at University College London Hospital (UCLH).
Dr Nilima Parry-Jones presents a podcast on the Guidelines for diagnosis and management of hairy cell leukaemia (HCL) and hairy cell variant (HCL-V). Hairy cell leukaemia (HCL) is an uncommon, chronic B cell leukaemia, first reported as a distinct entity in the 1950s.1, 2 HCL accounts for 2% of lymphoid leukaemias, with a male predominance and median age at diagnosis of 58 years. Classical HCL and its variant form (HCL‐V) are now regarded as separate entities,3 with different cytological, haematological and immunophenotypic features. BRAF V600E mutation, present in virtually 100% of cases of classical HCL,4 is regarded as a disease‐defining event, and is absent in HCL‐V. Dr Parry- Jones discusses the guideline in three parts: Part 1: Clinical and laboratory features. Part 2: Diagnostic tests and differential diagnosis including the use of molecular genetics. Part 3: Therapeutics and response assessment. Dr Nilima Parry-Jones is a Consultant Haematologist at the Aneurin Bevan Local Health, Wales.
Dr Nimish Shah presents a short podcast on the BSH Guideline Frontline Management of Post-Transplantation Lymphoproliferative Disorder in Adult Solid Organ Recipient Patients. Dr Shah discusses the guideline in three parts: 1) PET scan and its role in PTLD 2) The management of PTLD 3) Potential new developments This document is an updated guideline and details the recommendations for the frontline management of adult patients with an established diagnosis of post‑transplant lymphoproliferative disorder (PTLD) following solid organ transplantation (SOT). PTLD represents a spectrum of disorders resulting from lymphoid or plasmacytic cell proliferations that occur as a result of immunosuppression following SOT. In adult SOT recipients, PTLD is the second commonest malignancy after skin cancer and is the commonest cause of cancer-related mortality1. The reported incidence varies according to patient age, transplant type and the degree of immunosuppression. Historically, PTLD has been reported to occur most frequently in the first year following transplantation. However, more recent reports suggest that PTLD occurring late (beyond the first year) may be at least as common2–5 Dr Nimish Shah is a Consultant Haematologist at the Norfolk and Norwich University Hospital NHS Foundation Trust.
Dr Rachel Rayment presents a podcast on the BSH The use of prophylactic factor replacement for children and adults with Haemophilia A and B. Coagulation factor replacement in people with haemophilia (PWH) A or B may be given either in response to a bleed [on‐demand (OD) therapy] or regularly to prevent bleeding (prophylactic therapy). Guidelines for prophylactic treatment of children and adults with severe haemophilia A (SHA) were produced by the United Kingdom Haemophilia Centre Doctors' Organisation (UKHCDO) in 2010, summarising the high‐level, evidence‐based studies of prophylaxis in boys and advising on the role of prophylaxis in adults with SHA.1 This guideline builds on the former, accepting the clear evidence of benefit of prophylaxis in children with SHA. It addresses the optimum use of prophylaxis in children and adults with haemophilia A and B and gives evidence‐based recommendations where appropriate. The guidance will be of value to healthcare professionals, laboratory scientists, patients and those with a responsibility for funding services. Dr Rachel Rayment is a Consultant Haematologist; Haemophilia and Thrombosis Centre Director as well as Clinical Lead for ITP at the Cardiff and Vale University Hospital Health Board.
Dr Kim Linton presents a podcast on the BSH Guideline on the investigation and management of follicular lymphoma. Follicular lymphoma (FL) is a heterogeneous disease. For many it is experienced as a chronic, relapsing, indolent condition with long overall survival (OS). Most people affected have advanced disease at presentation; symptoms may include B symptoms (i.e. fever, night sweats and weight loss), fatigue and the local mass effect of lymph node enlargement. However, many people are asymptomatic at presentation. Some people are observed without treatment according to a ‘watch and wait' policy (see section Management of patients with newly diagnosed FL). In contrast to this, over a period of many years, 20–30% of patients will die from refractory FL or following transformation of their disease to high‐grade lymphoma.1 Prognostic indices may help discriminate between risk groups (see section Prognostic factors in FL). Dr Linton discusses the guidelines four main focus areas: 1) Important changes on the use of PET scanning. 2) Advances in the upfront management of patients with asymptomatic and symptomatic advance stage disease. 3) High risk disease in high grade transformation. 4) Advances in the management of relapse with some horizon scanning of promising drugs in development Dr Kim Linton is Consultant Medical Oncologist at Christie NHS Foundation Trust and Clinical Senior Lecturer at the University of Manchester.
Peter Baker presents a short podcast on the BSH Guideline on Laboratory aspects of assays used in haemostasis and thrombosis. Peter Baker discusses the following: 1) Preanalytical variables 2) Calibration and control of assays including generation reference ranges 3) Assays involved in the investigation of a bleeding and thrombotic tendency 4) TTP and Molecular testing This guideline is intended to help clinical laboratories perform high quality valid assays for basic procoagulants and anticoagulants as part of a routine diagnostic service. Areas that overlap with or have been included in other BSH (https://b‐s‐h.org.uk/guidelines/) or United Kingdom Haemophilia Centre Doctors Organisation (UKHCDO)(http://www.ukhcdo.org/guidelines/) guidelines have been omitted, including guidance on: heparin‐induced thrombocytopaenia (HIT); lupus anticoagulant (LA) testing; D‐dimer assays; platelet function testing; diagnosis of von Willebrand disease (VWD); measurement of factor replacement in haemophilia A and B; monitoring of anticoagulants [vitamin K antagonists (VKA) and direct oral anticoagulants (DOAC)]; and global assays of haemostasis (e.g. TEG, ROTEM, thrombin generation). Peter Baker is a Clinical Scientist working at the department of Haematology at Oxford University Hospitals NHS Trust.
Dr Christopher Fox presents a podcast on the BSH Good Practice Paper: The prevention of central nervous system relapsed in diffuse large B- cell lymphoma. As Dr Fox highlights, this is rare event in clinical practice and is devastating for patients when it occurs as well as causes much anxiety amongst clinicians. There is a lack of robust evidence to clearly recommend which patients should receive CNS prophylaxis and how this should be delivered. The data are largely retrospective with a wide variation in selection criteria for which patients received prophylaxis, primary treatment regimen used and type of CNS prophylaxis given. Dr Fox discusses three areas useful in this Good Practice Paper: 1) The problem of central nervous system relapse. 2) Who should receive prophylaxis. 3) To discuss what the optimum CNS prophylaxis may be. Dr Christopher Fox is a Consultant Haematologist at the Nottingham University Hospital NHS Trust.
Dr Graeme Smith presents a short podcast on the BSH Guideline for the management of mantle cell lymphoma The management of chronic myeloid leukaemia (CML) has seen considerable change in the last several years. The objective of this guideline is to provide healthcare professionals with clear guidance on the investigation and management of CML in adults and children. Dr Smith discusses the guideline in three parts: 1) Review of existing international guidelines on the investigation and management of myeloid leukaemia and why in his view there is a focus for U.K. guideline 2) Structure of the guideline 3) Discusses in detail four of the key areas that the guideline makes recommendations in terms of management of myeloid leukaemia in the U.K. Dr Graeme Smith at the time of recording is recently retired Haematologist and Clinical Director of the Leeds Oncology Centre; Leeds NHS Trust.
Dr Paula Bolton Maggs presents the podcast on the Guidelines on the use of irradiated blood components. To provide healthcare professionals with clear guidance on situations when the use of irradiated blood components is indicated. The term ‘blood component' means the therapeutic constituents of human blood (red cells, white cells, platelets and plasma) that can be prepared by various methods (JPAC https://www.transfusionguidelines.org/red‐book/definitions). The multidisciplinary writing group developed evidence‐based clarification and practical guidance in clinical areas of ambiguity. Publications relating to patients of all age groups have been assessed. The guidance may not be appropriate in all patient situations and assessment of individual circumstances with the appropriate risk assessments and patient involvement may lead to alternative decisions. Dr Bolton Maggs discusses the guideline in three parts but also, stresses that the podcast is not a substitute for reading the guideline: 1) Background on the use of irradiation and new evidence on aetiology of transfusion - associated - graft - versus - disease. 2) Specific issues for Hodgins and non Hodgins lymphoma 3) Summary of the changes for paediatric transfusion Dr Bolton- Maggs has recently retired as a Consultant Haematologist and Director of Serious Hazards of Transfusion (SHOT).
Dr Simon Rule presents a short podcast on the BSH Guideline for the management of mantle cell lymphoma. The aim is to provide healthcare professionals with guidance on the management of patients with mantle cell lymphoma (MCL). Individual patient circumstances may dictate an alternative approach. This is an update of the guidance published in 2012 (McKay et al, 2012), incorporating new therapeutic options, including transplant data. Developments in pathology, in particular molecular pathology and use of positron emission tomography/computed tomography (PET/CT) scanning in staging and response assessment are now covered in a separate Good Practice Paper (McKay et al, 2018). Dr Rule discusses the following: 1) Diagnostic 2) Management 3) Changes from the previous guideline. Dr Simon Rule at the time of recording was a Consultant Haematologist at Plymouth Hospitals NHS Trust and Professor in Haematology at Plymouth University Peninsula School of Medicine and Dentistry.
Dr Sue Pavord presents a podcast on the BSH UK guidelines on the management of iron deficiency in pregnancy. Iron deficiency remains a significant problem for pregnant women in the UK. The objective of these guidelines is to provide healthcare professionals with recommendations for the prevention, diagnosis and treatment of iron deficiency in pregnancy and in the postpartum period. The guidelines update and replace the previous ones (Pavord et al, 2012). The prevalence of anaemia in pregnancy remains high. In order to minimise adverse outcomes, including use of blood transfusion, further research is required to define optimal management, as many current recommendations are not supported by high quality evidence. Dr Pavord discusses this guideline in three parts: 1) The prevalence and impact of Iron deficiency in pregnancy. 2) How to diagnose it. 3) How to manage it. Dr Sue Pavord is a Consultant Haematologist at Oxford University Hospitals and Associate Senior Lecturer in Medicine at St Edmund Hall.