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- Title
Diagnostic Challenges in Hemophagocytic Lymphohistiocytosis, a Rare, Potentially Fatal Disease: Two Case Studies.
- Authors
Ionescu, Marcela Daniela; Prajescu, Bianca; Taras, Roxana; Popescu, Nicoleta; Vidlescu, Ruxandra; Smarandoiu, Mihaela; Rosca, Loredana-Elena; Enculescu, Augustina; Berghea, Elena Camelia; Toma, Claudia Lucia
- Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a rare, and potentially fatal, syndrome, characterized by immune system dysregulation, with excessive activation of the macrophages and cytotoxic T cells. It can be classified into primary (genetic) and secondary (acquired) forms. HLH presents with fever, hepatosplenomegaly, cytopenia, and hyperferritinemia, with involvement of various organs. The initial symptoms of HLH are non-specific, but as, if untreated, it can progress rapidly to multiorgan failure, timely diagnosis is essential. We present here two cases of HLH in infants that illustrate the importance of early diagnosis and appropriate treatment, along with a short review of HLH.
- Subjects
HEMOPHAGOCYTIC lymphohistiocytosis; CYTOTOXIC T cells; MACROPHAGE activation syndrome; MACROPHAGE activation; MULTIPLE organ failure
- Publication
Journal of Clinical Medicine, 2024, Vol 13, Issue 6, p1643
- ISSN
2077-0383
- Publication type
Article
- DOI
10.3390/jcm13061643