Tom Butler

Consultant, Barts Health NHS Trust

Perhaps one of the most exciting jobs in medicine is liaison haematology. What is it, and why do practitioners regard it as interesting?

Liaison haematology is the bridge between the laboratory and the clinician. As well as identifying and treating blood disorders, we’re responsible for interpreting blood test results and communicating them effectively to GPs. In hospitals, we work with multiple healthcare professionals, advising on everything from blood clotting and transfusions to immune system disorders — from pregnancy and newborns to end-of-life care.

Liaison haematology must access all areas

The job has an appealing aspect of having to ‘access all areas.’ Liaison haematologists are busy bodies. On any day, we can get a phone call from maternity one minute and then, the next, have to ensure the right blood goes to a patient who needs a liver transplant. We’ve got an important part to play right across the board in the functioning of a good hospital.

Flexible roles from lab to bedside

Haematologists are both laboratory specialists and clinicians — we have to pass the exams of both the Royal College of Pathologists and the Royal College of Physicians. We fill a gap between specialists and generalists.

These days, there’s a tendency to become more and more specialist, but you also need people who can use their specialist training to help diagnose and treat people — whatever the problem is. That’s what liaison haematologists do. We work across boundaries with GPs, nurses, healthcare scientists and other specialists — and there’s a real joy to walking in other people’s shoes. There’s an educational element to it, too.

Interpreting blood films and why it’s important

When patients have a blood test, computers quickly generate blood counts. If there’s something unusual, the liaison haematologist steps in, often looking at blood films under a microscope and detecting unexpected diseases such as leukaemia.

Part of our role is understanding how those laboratory tests are performed; the possible pitfalls; how errors can occur and then interpreting those results in a meaningful way — considering a patient’s medical history and examination.

These days, GPs can usually communicate with liaison haematologists using web-based systems. Nine times out of ten, we can reassure them about the results. If not, we advise on what other tests might be useful or whether the patient should come and see us. Liaison haematology has often been overlooked by NHS job planners, but it is finally coming to be recognised as a role that lies at the heart of good medical care.

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UK Sepsis Trust Founder & Joint CEO Dr Ron Daniels calls for a new approach to Infections Management, to tackle sepsis deaths and threat of antimicrobial resistance (AMR).

Sepsis is the final common pathway to death from most infectious diseases worldwide according to the WHO – the agency which also report that in 2019 an estimated 5 million deaths worldwide were attributed to antimicrobial resistance (AMR). Sepsis most commonly complicates bacterial infections such as UTIs and pneumonia, but can also complicate viral infections including COVID-19 and Ebola.

A complex interrelationship

Sepsis is a life-threatening condition in which the body’s response to an infection causes organ damage. In 2020, it was reported to claim an estimated 11 million lives globally among 49 million people affected every year – for context (and without diminishing the impact of the pandemic), in the more than three years to March 2023, COVID-19 claimed 6.9 million lives.

Antimicrobial resistance is causing harm to patients in our hospitals including in developed countries today, rather than it being a perceived future threat. However, it is not antimicrobial resistance in and of itself which brings about harm, but rather the infections which are rendered untreatable. The final common pathway to death, whether an infection is readily treated with antibiotics or not, is sepsis.

Therefore as the spectre of antimicrobial resistance grows, so will the death toll of sepsis. Simple infections which do not respond to antimicrobials will become life-threatening. Perhaps perversely, efforts to improve outcomes from sepsis which centre around heightening public awareness, resilient healthcare systems and health professional education might increase consumption of antimicrobials which might itself fuel AMR – thankfully data from the UK, Ireland and the USA suggest that this unintended harm of large-scale sepsis improvement efforts is not the reality.

Sepsis most commonly complicates bacterial infections such as UTIs and pneumonia, but can also complicate viral infections including COVID-19 and Ebola.

A new approach to Infections Management

Several governments around the world have initiated, and to varying degrees acted upon, national action plans regarding AMR. Whilst this progress is important, it isolates but one facet of the problem of infections management. We must improve the way in which we use antimicrobials in humans to treat and prevent disease, as well as in agriculture.

Dame Sally Davies has reminded us that AMR presents a more immediate threat to humanity than climate change. Addressing this existential threat requires a similarly coordinated approach.

If we are to envisage a future in which we can reasonably expect that our infections remain treatable, we have to change the paradigm with which we approach infection. Infections Management must address with equal figure four pillars: outbreak surveillance and pandemic preparedness, infection prevention and control, antimicrobial stewardship and the rapid recognition and treatment of sepsis. Until we do so robustly, we risk our existence.

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Lymphoma develops when white blood cells, called lymphocytes, grow out of control. Lymphocytes are part of the lymphatic system, which helps to fight infection.

“I hadn’t even heard of lymphoma before.” I have heard this statement many times in my work with Lymphoma Action, supporting people affected by the UK’s fifth most common cancer. When reflecting on their diagnosis, many people tell me how their initial response to being told they had lymphoma was a mixture of shock and confusion in learning that it is a type of blood cancer.

Lymphoma causes and symptoms

There are no known causes of lymphoma, and the symptoms can be quite general — making it tricky to spot. Common symptoms include swollen lymph nodes (commonly felt as lumps in the neck, armpit or groin); unexplained weight loss; drenching night sweats; itching without a rash; fatigue and fevers; difficulty getting over infections.

Lymphoma is a complex condition with over 60 different subtypes.

As these symptoms can also occur in other illnesses, having one or more doesn’t necessarily mean someone has lymphoma. However, it is important to be aware and always contact a GP with any concerns.

The most common type of blood cancer

Lymphoma is a complex condition with over 60 different subtypes. Treatment, therefore, depends on the type of lymphoma they have, its ‘stage’ and the general health of the person affected. Most treatments aim to send the lymphoma into remission.

Every 26 minutes, someone is diagnosed with lymphoma in the UK. It is the most common cancer in teenagers and young adults aged 15 to 24. Yet, 66% of people recently surveyed through the Charity Awareness Monitor did not know that lymphoma is the most common type of blood cancer.

September is Blood Cancer Awareness Month, and we, at Lymphoma Action, aim to raise awareness of the condition through our ‘Let’s talk lymphoma’ campaign. It is hard enough being told that you have cancer, but when it is little-known and difficult to explain to others, it only adds to the challenges of a diagnosis. We hope to open up the conversation about lymphoma and invite everyone to join us in raising awareness so that we can help more people.  

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New scientific developments are making what once seemed impossible possible for people living with this serious disease.

The last 30 years have brought dramatic advances in human health through the discovery of new medicines; people now live with HIV, cancers are being cured and the risk of developing heart disease can be significantly reduced.

But as companies look to the future to drive new developments in human health, they tend to focus working on diseases that impact a specific organ — like the lung — or they focus on one type of therapeutic approach.

Walking a different path

Our goal is to create transformative medicines for people with serious diseases. To achieve this, we target serious diseases where we have strong insight into the biological mechanism and where there is significant need for new treatments. It is only once we have fully understood the biological problem we are trying to solve that we invent, partner or acquire a potential therapeutic solution. We believe this approach maximises our chances of success of translating science and research into medicines for patients who need them.

Sickle cell – the ‘first molecular disease’

Sickle cell is a serious, life-threatening inherited disease affecting the red blood cells. People who have sickle cell are frequently anaemic and can suffer from regular sickle crises. Crises occur when the red blood cells block blood vessels resulting in severe and debilitating pain which can happen anywhere in the body at any time. This has a significant impact on their life; people who have more sickle crises per year are more likely to report high impact on their daily activities, school attendance, job retention, family and social life, and stress levels.

Yet, despite sickle cell being described as the ‘first molecular disease’ in 1949, the current treatment landscape has remained focused on addressing symptoms of the disease rather than the underlying cause.

Deep understanding of the mechanisms of disease allows us to focus our resources into potential solutions to biological problems.

Our approach to finding a treatment

At Vertex, our insight into how to treat the disease comes from natural variations in the DNA of a small number of people with sickle cell. Normally, the symptoms of sickle cell would begin approximately three months after birth as the haemoglobin in red blood cells changes from foetal haemoglobin to adult haemoglobin. However, in some people, this switch never happens. Studies have shown us that people with sickle cell who continue to produce foetal haemoglobin have few or no symptoms of their disease.

Scientists at Vertex therefore hypothesised that targeting the processes in our body that enable foetal haemoglobin to be produced again could provide a possible therapeutic solution for people with sickle cell. Clinical trials are ongoing to validate this hypothesis to potentially create treatments to improve the lives of people living with this disease. 

Translating science into commitment

Deep understanding of the mechanisms of disease allows us to focus our resources into potential solutions to biological problems. And we take this seriously, investing approximately 70% of our operating expenses into research and development year on year. But on top of science and drug discovery, we also have a commitment to partnership with all stakeholders as we look to bring treatments to people living with sickle cell.

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September 2022

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The symptoms of blood cancer are many and varied – some might even say vague. Often, patients and medical professionals alike put them down to age, stress or other less serious conditions.

It is found that 30% of people who get a blood cancer diagnosis have to see their GP three or more times. Another 30% are diagnosed after a visit to A&E.

Diagnosing blood cancer early can make all the difference to people’s mental health, treatment options and chances of survival. So, it’s important for everyone to be aware of the main symptoms.

Know the symptoms of blood cancer

The signs and symptoms of blood cancer may include:

• Unexplained weight loss
• Unexplained bruising or bleeding
• Lumps or swellings
• Shortness of breath (breathlessness)
• Drenching night sweats
• Infections that are persistent, recurrent or severe
• Fever (38°C or above) that is unexplained
• Unexplained rash or itchy skin
• Pain in your bones, joints or abdomen (stomach area)
• Tiredness that doesn’t improve with rest or sleep (fatigue)
• Paleness (pallor).

Symptoms in different skin tones

It’s also important to recognise that many sources of information, including medical handbooks, focus on how symptoms present in white skin only. This is misleading.

On black and brown skin, bruises can be difficult to see initially, but as they develop, they show as darker than the skin around them. Rashes look purple or darker than the surrounding skin. Paleness or pallor (caused by a lack of red blood cells) can be easier to spot in the palms, lips, gums, tongue or nail beds or by pulling down the lower eyelid — if it’s white or pale pink inside rather than red, it’s a sign of pallor.

Diagnosing blood cancer early can make all the difference to people’s mental health, treatment options and chances of survival.

Report anything unusual

The most important message is for people to contact their GP if they have any symptoms that are long-lasting or unusual for them. If symptoms carry on or get worse after seeing the GP, make sure to return. Kerry Williams was diagnosed with myeloma last year. She went to her doctor because she had a period that went on longer than normal. “If I’d just ignored the fact that my period was going on for too long, at what point would it have been discovered? Because it was picked up early, it hasn’t affected my kidneys or bones. So, if you don’t think something’s right, then definitely get it checked.”

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T’sharne is one of the 15,000 people in the UK living with a sickle cell disorder (SCD), the term used to describe a range of inherited blood disorders affecting the haemoglobin in red blood cells.

16-year-old T’sharne just wants people “to understand that sickle cell is athing — that it exists,” and to understand the impact it can have upon his day-to-day life. T’sharne has the most serious form of SCD, known as sickle cell anaemia.

Due to the geographical origins of SCD, most people affected are of African or Caribbean backgrounds, although anyone can have it — regardless of their ethnicity.

Understanding the condition

SCD is an inherited condition. If both parents carry the sickle cell gene, there is a one in four chance of their child having sickle cell. It qualifies as a rare genetic illness (defined by the European Union as one that affects fewer than five people in every 10,000), and its impact on an individual’s life — and their family and friends — is significant.

T’sharne’s mother Clementina, a teacher and campaigner, says that finding out T’sharne had sickle cell was initially difficult for her to accept and she felt guilty for ‘giving’ him the condition. “Despite having sickle cell in our family, neither I nor my husband were aware that we had the sickle cell trait or of the risks involved when having a child.”

Symptoms of sickle cell disorder

The main symptoms of sickle cell disorder are anaemia and episodes of severe pain, known as a sickle cell crisis. Pain occurs when red blood cells change from a round, doughnut-like shape to sickle-shaped, inhibiting the flow of oxygen around the body and causing the cells to stick together, resulting in blockages in small blood vessels.

People with sickle cell are at risk of several complications including leg ulcers, sight loss and a serious condition called acute chest syndrome (when blood flow to the lungs is blocked).

It was a chest crisis that first saw T’sharne receive donated blood at only 10 years old; he now receives regular blood transfusions every four weeks via a process called Exchange Blood Transfusions (EBT). It is a highly successful treatment where up to a third of a patient’s blood is replaced with blood from healthy donors.

SCD is an inherited condition.

Importance of ethnically matched blood

Since starting on regular blood transfusions in 2018, T’sharne has had no emergency admissions to the hospital — a remarkable achievement given he had previously missed a whole school term due to his illness.

Clementina and T’sharne support the Sickle Cell Society’s Give Blood, Spread Love project, which raises awareness of the need for more African and Caribbean heritage blood donors. Transfused blood needs to be ethnically matched to avoid further health complications, and there is an ongoing challenge to encourage more Black and mixed race donors to come forward.

Since joining the project, Clementina has become a regular blood donor (most people with the sickle cell trait can donate blood) and has found out she has a rare subtype of blood, known as Ro, which is 10 times more common in people with African and Caribbean heritage and is especially needed to provide matched blood to people with sickle cell.

Clementina has become a powerful advocate for blood donation, encouraging her friends, family and colleagues to donate.

How important is blood donation?

Clementina and T’sharne agree that more awareness and understanding of sickle cell is needed to support those with SCD and their families. While being a strong advocate for a holistic approach to care, they also believe that more funding is needed to increase the range and accessibility of treatments available for sickle cell, including making the only current cure — a stem cell transplant — more widely available. The Sickle Cell Society needs people from all communities to help raise awareness of sickle cell and the lifesaving impact of blood donation on those affected.

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Dr Michael J Weinbren

Consultant Medical Microbiologist and Infection Control Doctor, NHS

Bruce Caldwell

Country Business Leader for Integrated Diagnostic Solutions, UK & Ireland, BD

Diagnostic tests are playing a critical role in the fight against sepsis and bloodstream infections and enabling clinicians to give patients the correct antibiotic treatments for recovery.

Reviews of blood culture practices, new technologies and antimicrobial stewardship are seen as critical steps to support the management of bloodstream infections, such as sepsis. Closer partnerships between industry, government, infection control experts, NHS policymakers and frontline staff are also important in the fight against such infections, according to experts in the field.

Sepsis is a life-threatening reaction to infection and happens when your immune system overreacts and starts to damage your body’s own tissues and organs. It affects 245,000 people and claims 48,000 lives a year in the UK. An NHS England (NHSE) review is currently looking to improve antibiotic prescribing and patient safety to improve outcomes from sepsis.

Hidden pandemic

Access to diagnostic tools and processes that ensure blood samples are correctly taken and tested within a designated timeframe is pivotal in tackling sepsis.

Bruce Caldwell, Country Business Leader for Integrated Diagnostic Solutions (UK and Ireland) for global medtech supplier BD (Becton, Dickinson and Company), reinforced the importance of industry–healthcare collaboration, explaining: “The correct use of accurate diagnostics enables bloodstream infections to be detected and allows microbiologists and physicians to determine the right antibiotic to use. Late intervention could result in poor patient outcomes at increased costs.”

The NHS conducts millions of blood-related tests, and this delivers rapid treatment for patients but also makes the best use of antimicrobials at a time of a ‘hidden pandemic’ of antimicrobial resistance.

Late intervention could result in poor patient outcomes at increased costs.

Bruce Caldwell

Early detection

BD continues to advance the world of health efficiently, safely, and sustainably by applying its heritage and broad portfolio to support healthcare systems across the UK and Ireland. The organisation manufactures a range of diagnostic and medical devices which are used daily within the NHS; these include BD Vacutainer® blood collection devices, BD FACSLyric flow cytometers and BD BACTEC blood culture instruments to diagnose and determine treatments.

Pathway review

Blood cultures expert and Consultant Medical Microbiologist Dr Michael Weinbren is working with the NHSE blood culture pathway review to deliver key performance indicators for better management of patients with sepsis.

Having optimised blood culture pathways at Chesterfield Royal Hospital, he was involved in the 2018–2019 survey, which found clear room for improvement in blood culture practices. Inconsistencies were found in the three phases of the pathway: collection and transportation of the specimen, laboratory processing and the post-analytical phase used to determine treatment.

Only 3% of UK sites analysed blood cultures within four hours of collection. In 40% of cases, it took more than 24 hours — enough time for some specimen organisms to die and affect readings — and 87% of sites took one set, rather than the recommended two sets, of blood.

With 20% of bloodstream infection or sepsis patients on ineffective antimicrobial therapy, Weinbren notes that speeding up testing would mean patients are quickly prescribed the correct narrow-spectrum antibiotics, improving antimicrobial stewardship.

Weinbren says failure to act and optimise the blood culture pathway will see a “vicious spiral” of increasing mortality from sepsis and fewer effective antibiotics by 2050.

Heightened awareness

Dr Ron Daniels, Executive Director of the UK Sepsis Trust and Vice President of Global Sepsis Alliance, believes thousands of deaths could be avoided with a heightened awareness of sepsis and rapid treatment.

“We must ensure people access healthcare at the right time, empower health professionals to act and better integrate diagnostics into clinical systems.

“Placing such technologies closer to the patient, rather than in centralised laboratories, will enable clinicians to respond rapidly and accurately, saving patients’ lives and slowing the spread of antimicrobial resistance,” he adds.

BD BACTEC Instruments and the BD FACSLyric flow cytometer are in-vitro diagnostic medical devices bearing a CE mark.

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Dr Fenella Willis

Consultant Haematologist, St George’s University Hospitals, Trustee Myeloma UK

The pandemic brought uncertainty, delays and disruption in care/access to treatments for patients with a type of blood cancer — myeloma. During the pandemic, society became more aware of this vulnerable group. But as we have discarded our masks and restrictions — what does their future look like?

Myeloma is difficult to diagnose due to vague/common symptom presentation (eg. fatigue, back pain, infection).

Impact on diagnosis

During the pandemic, the entire cancer pathway was disrupted (GP access, diagnostic pathways, hospital services), which has had a significant impact on myeloma diagnosis. In 2020–2021, over 500 fewer patients were diagnosed, and patients presented late with irreversible life-changing complications (eg. spinal fractures, renal failure) resulting in increased morbidity and mortality.

Delays in diagnostic pathways resulted in a surge of activity after the lockdowns eased, increasing pressures on already overstretched NHS services. For myeloma patients, maintenance of strict infection control measures, streamlined care pathways and digital services have become the norm. Clinicians must challenge decisions on services provided to these vulnerable patients to reverse the negative impact of delayed diagnosis/treatment.

Ongoing infection risk

Myeloma patients have an eleven-fold increased infection risk and eighteen-fold risk of viral infection in the first year after diagnosis due to the immunosuppression both by the disease and treatment. Infection risk continues throughout the patient’s lifetime.

Prophylactic antibiotics used in the first 12 weeks of treatment significantly reduce the risk of febrile episodes and death from infection. Reduction in viral infection risk requires vaccination and antiviral treatments. Even with intervention, patients may not always respond optimally. Between January and March 2022, data from the Office of National Statistics showed that one in twenty-two patients who died with Covid-19 in England and Wales had blood cancer.

The clinician-patient conversations regarding easing restrictions and risks of infections with the ability to sustain a good quality of life are essential.

Priorities for patients

For blood cancer patients, it is paramount to ensure speedy vaccine rollout, timely access to Covid-19 treatments and rapid testing and fast approval of new drugs/treatments.

The clinician-patient conversations regarding easing restrictions and risks of infections with the ability to sustain a good quality of life are essential. Access to vaccination and treatments has helped, but careful consideration of potential risk and the use of masks in healthcare environments and crowded areas are also vital. Legislation for improved employment protection and development of safe-working environments remains essential for patients to remain healthy in working environments.

Impact on mental health

The last two years have seen significant increases in mental health disorders, and 72% of patients highlighted that myeloma had a moderate–high impact on their quality of life. Additionally, 30% reported a major impact on their mental health, rising to 40% when looking at the BAME (Black, Asian and Minority Ethnic) community. As services return to normal, these patients need enhanced psychological support and access to specialist medical and nursing support.

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Sickle cell disorder mainly affects people from African and Caribbean backgrounds. It’s an inherited blood condition that can shorten life and lead to severe unpredictable bone pain which blights many individuals’ lives.

How would you feel if from a young age, you knew what you were likely to die from? If you had witnessed friends or relatives die young from the same illness? If your life and routine were frequently punctuated by episodes of severe pain? Angry? Frightened? Depressed? Desperate? Probably a combination of all of these. How about if the services the NHS offered you were patchily distributed, under-resourced and in some cases, frankly inadequate?

Sadly, this is the case for many patients in the UK with sickle cell disease.

A disease affecting any bodily system

Very strong painkillers can be required when someone is suffering what’s known as a sickle cell crisis. In addition to the pain, sickle cell disease can affect almost every organ in the body, from strokes in childhood to kidney, lung and liver failure in later life.

It is the most common inherited blood disorder – more prevalent than cystic fibrosis. Around 15,000 people have it in the UK. But the funding for sickle cell, which mainly affect Black people, is dwarfed by the amount spent on other inherited diseases affecting Caucasian populations.

Sickle cell disease can affect almost every organ in the body, from strokes in childhood to kidney, lung and liver failure in later life.

Addressing variations in care

Although there are dedicated teams of doctors, nurses and psychologists delivering good care to those with sickle cell disease, services for this group of patients are still underfunded and neglected.

So, what can be done? Most importantly, greater investment is needed in services for patients, in education about the condition for NHS clinicians and in research into new and better treatments. Some patients get good NHS treatment, but for others, it can be poor. We have to overcome that variation.

All 42 Integrated Care Systems (ICS) around the country should commission sickle cell services so that there is a uniformly high standard of care delivered everywhere. Hospital trusts need appropriate facilities where specialists can look after patients and have enough funding to employ those specialist doctors, nurses and other staff to do so.

Potential treatment options

There is an increasing range of potential treatments for sickle cell disease; new drugs with the potential to ameliorate the disease; stem cell transplants; and further down the line, hopefully, gene therapies with a potential to cure this dreadful disease. There are lots of other ways the NHS can do better for sickle cell patients. For example, making it free for all of them to get prescriptions.

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Accelerating progress in leukaemia diagnosis, treatment, care and research has the power to both save and improve lives.

Leukaemia is a difficult disease to treat. It occurs when white blood cells develop in an uncontrolled way, dramatically reducing their ability to fight infection, but there is hope.

Acute leukaemias are fast developing and particularly hard to treat. Current treatments for acute myeloid leukaemia (AML) are incredibly harsh on the body, and the five-year survival rate is still devastatingly low. Stem cell transplantation is a life-saving treatment and is currently one of the only effective options available. Yet, patients will often relapse following a stem cell transplant.

Preventing leukaemia relapse

Dr Pramila Krishnamurthy is researching how relapse can be prevented through exploring the possibility of using immune cells from the stem cell donor and giving these to the patient after their original transplant. She says: “When patients relapse or fail to respond to treatment, it’s heart-breaking – especially when we only have limited treatment options in this scenario.”

Dr Krishnamurthy’s research may be able to help correct a patient’s immune response, eliminating the remaining leukaemia cells and preventing relapse.

Stem cell transplantation is a life-saving treatment and is currently one of the only effective options available.

Developing new treatment methods

Encouragingly, research breakthroughs are happening all the time. Some of these include completely new ways of treating leukaemia, like Dr Konstantinos Tzelepis’ important discovery during his Leukaemia UK John Goldman Fellowship in 2021.

Looking at a particular protein, METTL3, and its role in how leukaemia cells develop, Dr Tzelepis investigated whether inhibiting the action of one particular protein could eliminate leukaemia – with minimal side effects. In doing so, Dr Tzelepis and his group at the University of Cambridge identified a new targeted drug with the potential to treat AML. 

Dr Tzelepis says, “This is the result of many years of research and could mean the beginning of a new era for cancer therapeutics. We are at an exciting stage with clinical trials about to begin. The first AML patients to access the drug will be adults who haven’t responded well to current treatment options such as chemotherapy. If the results are as expected, we also hope the drug will offer an important treatment option for childhood AML.”

New hope for the future of treatment

Dr Tzelepis’ breakthrough has the potential to provide a vital new treatment option for AML patients. Leukaemia UK is funding work like this to bring hope for kinder and more effective treatments to those who receive this diagnosis and crucially, to stop leukaemia from devastating lives.

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