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- Title
Does your unwell patient have haemophagocytic lymphohistiocytosis?
- Authors
Holloway, Amelia; Ahmed, Saad; Manson, Jessica J
- Abstract
Haemophagocytic lymphohistiocytosis is a severe systemic hyperinflammatory syndrome characterised by dysregulation of immune cells and excessive production of cytokines, also known as a cytokine storm. It has distinctive clinical features with fever, hyperferritinaemia and falling blood counts. In adults, this usually occurs secondary to an underlying driver or trigger including infection, malignancy or rheumatic diseases. Prompt treatment with immunomodulatory therapy, including corticosteroids and the recombinant IL-1 receptor antagonist anakinra, is recommended to switch off the cytokine storm. Etoposide-based regimens are sometimes needed, and newer therapies such as emapalumab and JAK inhibitors are increasingly being used. The incidence of haemophagocytic lymphohistiocytosis has increased significantly over the last 20 years which may partly reflect increased awareness of the condition. Although relatively rare, haemophagocytic lymphohistiocytosis can be encountered by a broad range of hospital physicians, so knowing how to diagnose and treat this condition is essential. This article reviews the pathogenesis, clinical features, causes, diagnosis and treatment of haemophagocytic lymphohistiocytosis to improve physician recognition and management of this condition to improve future patient outcomes.
- Subjects
UNITED Kingdom; EDUCATION of physicians; THERAPEUTIC use of monoclonal antibodies; ADRENOCORTICAL hormones; HEMOPHAGOCYTIC lymphohistiocytosis; IMMUNE system; TREATMENT effectiveness; JANUS kinases; PROFESSIONS; IMMUNOLOGIC receptors; RECOMBINANT proteins; CYTOKINES; EVIDENCE-based medicine; NEUROTRANSMITTER uptake inhibitors; MACROPHAGE activation syndrome; INTERLEUKIN-1; DISEASE risk factors; CHEMICAL inhibitors; SYMPTOMS; ADULTS
- Publication
British Journal of Hospital Medicine (17508460), 2024, Vol 85, Issue 3, p1
- ISSN
1750-8460
- Publication type
Article
- DOI
10.12968/hmed.2023.0394