New resources to support more clinicians to recognise the rare and devastating condition HLH – in turn helping to improve patient care and outcomes – have been developed by the Getting It Right First Time (GIRFT) team.
GIRFT has led a collaborative, cross-specialty group of clinicians towards the first consensus pathway for the little-known condition of haemophagocytic lymphohistiocytosis (HLH), with the aim of raising awareness among acute care providers so that HLH is considered more often as a diagnosis. If diagnosed, the pathway and its accompanying guidance can be used to steer clinical teams through the patient’s treatment and onward support.
The resources – co-badged by the British Society for Rheumatology and patient support group Histio UK – showcase consensus recommendations for care across the patient pathway, as well as best practice case studies from teams who have significantly reduced their mortality rates.
HLH is a devastating syndrome with uncontrolled activation of the immune system leading to hyperinflammation, tissue damage and multi-organ failure. With symptoms often mimicking sepsis, it affects people of all ages and has a mortality rate of around 50%. While considered a rare disease, cases are increasing and it is thought to be underdiagnosed.
The syndrome can be triggered by infection (including COVID-19), malignancy, autoimmune/autoinflammatory disease, pregnancy, some drugs, CAR-T cell therapy for cancer treatment and bone marrow transplant. As such, patients can present in any area of acute medicine and treatment requires cross-specialty management.
While GIRFT’s work has been led by rheumatologists, the pathway, guidance and webinar are equally relevant for colleagues in emergency medicine, haematology, general medicine, critical care, paediatrics, immunology, rare diseases, infectious diseases/virology/microbiology, obstetrics and pharmacy, as well as operational colleagues and commissioners.
“Patients can present with HLH in any part of acute medicine, so increasing awareness across the system can mean earlier recognition and treatment and potentially more lives saved. We hope these resources will start a national clinical conversation about HLH among all providers of acute care, so that the syndrome is considered - and hopefully ruled out - as soon as possible.”
Webinar recording
To coincide with Histiocytosis Awareness Month (September), GIRFT held a webinar to discuss and consider any questions on the new HLH pathway and guidance.
Click below to watch the recording: Working together to improve care and outcomes for people with suspected haemophagocytic lymphohistiocytosis (HLH)
