Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening condition that is characterized by an overactive response of the immune system with excessive production of proinflammatory cytokines. Initial presentation of this condition often mimics and overlaps with many diseases including infections, sepsis, and multiorgan failure syndrome, which makes diagnosis the diagnosis of HLH challenging. Herein is described a case of a patient who developed acute respiratory distress syndrome and multiple organ failure related to HLH in a setting of probable viral pneumonia. The diagnosis was established based on laboratory and bone marrow biopsy findings. This patient was treated with the standard chemotherapy regimen of intravenous dexamethasone, etoposide in addition to intrathecal methotrexate for central nervous system involvement.
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http://dx.doi.org/10.1177/23247096211052180 | DOI Listing |
Background: Anakinra is an interleukin-1 receptor antagonist (IL-1Ra). Since IL-1 has been shown to play a key role in the etiology of different autoinflammatory diseases, blocking its pathway has become an important therapeutic target, even in neonates.
Aims: We aimed to report our experience in using anakinra to treat specific neonatal inflammatory conditions.
Cureus
December 2024
Internal Medicine, Centro Hospitalar e Universitário de Coimbra, Coimbra, PRT.
Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal hyperinflammatory syndrome characterized by dysregulated immune activation and systemic inflammation. Secondary HLH is often triggered by infections, with being an infrequently reported cause. Peripheral axonal neuropathy is a rare and poorly understood complication of HLH.
View Article and Find Full Text PDFJ Mycol Med
January 2025
University of Illinois College of Medicine Peoria, Peoria, IL, USA; Children's Hospital of Illinois, Peoria, IL, USA. Electronic address:
Background: Histoplasmosis is the most prevalent endemic mycosis in the United States, typically affecting immunocompromised individuals. Diagnosis of histoplasmosis in immunocompetent patients is rare, particularly among young infants, with only a few cases reported.
Case Presentation: We present a 4-month-old female with a history of prematurity who initially presented with 11 days of fever.
Langerhans cell histiocytosis (LCH), juvenile xanthogranuloma (JXG) family lesions, and Rosai-Dorfman-Destombes disease (RDD) are now classified by the World Health Organization (WHO) under the heading of histiocytic/dendritic cell neoplasms. Each disease may manifest as a focal lesion, as multiple lesions, or as a widespread aggressive systemic disease with visceral organ involvement. Erdheim-Chester disease (ECD) is a rare systemic disease process of adults with limited cases in children.
View Article and Find Full Text PDFEur J Case Rep Intern Med
December 2024
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.
Case Reports: We discuss two intensive care unit (ICU) cases illustrating the diverse manifestations of HLH and the critical importance of early recognition and treatment.
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