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 common^2–5

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.