Abstract Background/Aims Haemophagocyticlymphohistiocytosis (HLH) is a life-threatening, rare disorder characterised byoveractivation of the immune system, resulting in fever, cytopenia and hyperferritinaemia.Mortality is up to 50% due to nonspecific symptoms and delayed diagnosis.Recent guidelines published by Getting It Right First Time (GIRFT) suggest thathigh ferritin alerts could accelerate diagnosis and consequently reducemortality. This service evaluation aims to determine whether patients at QueenElizabeth Hospital, Birmingham (QE) with a high ferritin had HLH and if theywere managed according to new GIRFT guidelines. Methods A retrospective service evaluation of patients admitted to the QE in 2023 with a ferritin 9999 was carried out to identify if these patients could have been diagnosed with HLH, using the H score. Further data on demographics, investigations, management and outcomes were collected. Results During the allocated timeperiod, 76 patients were admitted to the QE with a ferritin greater than 9999.Of these patients, 46 (60.5%) were alive on discharge while 30 (39.5%) died. Incalculating the H score, 25 patients reached the threshold of 169 points, indicating a high likelihood (90%) of HLH. Only nine of these patients werediagnosed, though it was suspected in an additional four, leaving 12 (48%)patients with a potentially missed diagnosis. Of those diagnosed, five (55.6%)died and of those with an H score 169, 11 died. Reassuringly, all patientswho were diagnosed with HLH, with and without biopsy findings, were given anakinra and most (77.7%) received concurrent steroids, with other patientsreceiving rasburicase (22.2%), brentuximab (11.1%) and etoposide (11.1%). Thecause of HLH in four was infection, four had a haematological malignancy andone had polymyositis. Conclusion These results indicate that HLH should be considered in severely unwell patients. Mortality rates aligned with published data, reinforcing the importance of timely diagnosis. We recommend integrating a local ferritin alert, a formal HLH MDT and pre-populated HLH investigation bundle to support early recognition and treatment and identify potentially undiagnosed patients. Disclosure K. Middleton: None. A. Lahiri: None. S. Byravan: None. N. Narayan: None. P. Jobanputra: None. E. Justice: None.
Middleton et al. (Wed,) studied this question.