Adult hemophagocytic lymphohistiocytosis is a lifethreatenning disease which has hepatic manifestations mimicking acute hepatitis or can present with fulminant hepatic failure. The undelying pathophysiology is immune dysregulation causing a hyperinflammatory state. Clues to diagnosis include extremely high ferritin levels, whereas definitive diagnosis is usually made by bone marrow, as opposed to liver biopsy. Even with early and appropriate treatment with weekly dexamethasone and etoposide, mortality remains high.
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Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare complication of multiple myeloma (MM), with limited data available on its incidence, clinical presentation, and treatment. The underlying mechanisms linking MM and HLH remain unclear, including the potential role of MM treatment agents in triggering HLH.
Methods: This case report presents a patient with MM who developed HLH while on lenalidomide maintenance therapy.
Macrophage Activation Syndrome/Secondary Hemophagocytic Lymphohistiocytosis in Adult-Onset Still's Disease: An Uncommon Initial Presentation in a Young Nepalese Female: A Case Report.
Clin Case Rep
January 2025
Department of Rheumatology, Institute of Medicine Tribhuvan University Maharajgunj Kathmandu Nepal.
Hemophagocytic lymphohistiocytosis (HLH), is a fatal systemic hyperinflammatory syndrome. HLH may be due to immunosuppression, infections, cancer, or autoimmune diseases with fever and cytopenia. HLH which occurs in adult-onset Stills disease (AOSD) is called secondary HLH, also known as macrophage activation syndrome (MAS).
View Article and Find Full Text PDFEfficacy, safety, and target engagement of dazukibart, an IFNβ specific monoclonal antibody, in adults with dermatomyositis: a multicentre, double-blind, randomised, placebo-controlled, phase 2 trial.
Lancet
January 2025
Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA. Electronic address:
Background: Dermatomyositis is a chronic autoimmune disease with distinctive cutaneous eruptions and muscle weakness, and the pathophysiology is characterised by type I interferon (IFN) dysregulation. This study aims to assess the efficacy, safety, and target engagement of dazukibart, a potent, selective, humanised IgG1 neutralising monoclonal antibody directed against IFNβ, in adults with moderate-to-severe dermatomyositis.
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View Article and Find Full Text PDFPrognostic Impact of Aetiology in Adult Hemophagocytic Lymphohistiocytosis: Insights from an Intensive Care Unit Experience.
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Intensive Care Unit, Pedro Hispano Hospital, Matosinhos Local Health Unit, Matosinhos, Portugal.
Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome marked by excessive immune activation. It can be triggered by various factors, including infections, malignancies, and autoimmune diseases, making the diagnosis challenging due to its overlap with other severe conditions.
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