Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening immune activation syndrome that should be recognized earlier for effective treatment. Adults usually have secondary HLH. An uncommon cause of secondary HLH is AIDS and simultaneous opportunistic infections. Acute human immunodeficiency virus (HIV) and opportunistic infections are also independent causes of HLH, so the presence of both should raise suspicion, especially if patients fulfill the criteria. HLH secondary to severe babesiosis is a rare entity as well. Some patients might not meet the full criteria of HLH on presentation, especially when some specific lab test results are still pending. A delay in diagnosis can happen in those cases. Here, we present two cases. The first case is of a 35-year-old homosexual male who presented with constitutional symptoms of one-week duration. He was diagnosed and started on the treatment of HIV. His fever was not resolving and further investigations led to a diagnosis of disseminated histoplasma infection. The patient fulfilled the criteria of HLH as well. Prompt therapy resulted in the improvement of clinical and laboratory parameters. The second case is of a 72-year-old female presenting with fever. A diagnosis of severe babesiosis and secondary HLH was made. Treatment of babesia resulted in the improvement of clinical and biochemical parameters.
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http://dx.doi.org/10.7759/cureus.17947 | DOI Listing |
Biomedica
December 2024
Facultad de Ciencias de la Salud, Universidad ICESI, Cali, Colombia, Departamento de Pediatría, Alergología e Inmunología Pediátrica, Fundación Valle del Lili, Cali, Colombia.
Introduction. Hemophagocytic syndrome is an under-recognized condition with high mortality in the pediatric population. It is characterized by excessive activation of immune cells and cytokine release, leading to persistent inflammation.
View Article and Find Full Text PDFBlood Adv
January 2025
The Ohio State University, Columbus, Ohio, United States.
Hemophagocytic lymphohistiocytosis (HLH) is a rare but aggressive and potentially lethal hyperinflammatory syndrome characterized by pathologic immune activation and excessive production of proinflammatory cytokines leading to tissue damage and multisystem organ failure. There is an urgent need for the discovery of novel targets and development of therapeutic strategies to treat this rare but deadly syndrome. Protein Arginine Methyltransferase 5 (PRMT5) mediates T cell-based inflammatory responses, making it a potential actionable target for the treatment of HLH.
View Article and Find Full Text PDFImmunotherapy
January 2025
Department of Internal Medicine, Division of Medical Oncology, Koc University School of Medicine, Istanbul, Türkiye.
Hemophagocytic Lymphohistiocytosis (HLH) is a severe and potentially life-threatening condition characterized by an excessive and uncontrolled activation of the immune system. ICI-related hemophagocytic lymphohistiocytosis (irHLH) is a rare immune-related adverse event with an incidence of 0.03% to 0.
View Article and Find Full Text PDFTurk J Pediatr
November 2024
Division of Pediatric Immunology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Türkiye.
Background: Griscelli syndrome (GS) is a rare genetic disorder characterized by oculocutaneous albinism and variable immune dysfunction. Among three distinct types of GS, occurring due to different genetic mutations; GS type 1 presents with neurological manifestations, hemophagocytic lymphohistiocytosis (HLH) generally develops in GS type 2, and GS type 3 primarily exhibits oculocutaneous albinism. HLH, a life-threatening condition with excessive immune activation, may occur secondary to various triggers, including infections, and develop in different tissues, as well as in the testis, similar to Erdheim-Chester disease.
View Article and Find Full Text PDFClin 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).
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