Retrospective single‐centre analysis of diagnostic approach to adult‐onset haemophagocytic lymphohistiocytosis. Issue 6 (21st June 2021)

• Record Type: Journal Article
• Authors: Lee, Wei‐I.; Talaulikar, Dipti; Cook, Matthew C.
• Is Part Of: Internal medicine journal. Volume 51:Issue 6(2021)
• Page Start: 939
• Page End: 947
• Publication Date: 2021-06-21
• Subjects: Lymphohistiocytosis -- ferritin -- macrophage activation; Medicine -- Periodicals; 616
• DOI: 10.1111/imj.14891
• Languages: English
• ISSNs: 1444-0903

Abstract

Abstract: Background: Haemophagocytic lymphohistiocytosis (HLH) is a rare disorder with a poor prognosis characterised by substantial immune activation leading to end‐organ failure. In childhood, genetic defects that impair cytotoxic function of natural killer cells and T cells. (HLH) are often identified. In adults, clinical manifestations are similar to those observed in children but the aetiology is often unclear. Aims: To evaluate whether poor prognosis for adult HLH is in part due to lack of awareness of the disorder, which results in incomplete investigation and failure to implement timely treatment. Methods: We performed a retrospective case series of adult‐onset HLH in a tertiary hospital in Australia. We evaluated clinical characteristics, treatment and outcome, and related these to application of standard diagnostic criteria for HLH. Results: In our centre, incomplete assessment of HLH criteria was common. Serum ferritin was the criterion most commonly assessed. Hyperferritinaemia ≥10 000 μg/L was highly sensitive in detecting patients with adult‐onset HLH; however, the majority of patients who had hyperferritinaemia ≥10 000 μg/L did not have adult‐onset HLH. Conclusion: The present study highlights the importance of comprehensive application of diagnostic criteria to improve accuracy and timelines of the diagnosis of adult onset HLH.