AI Article Synopsis
- Secondary hemophagocytic lymphohistiocytosis (HLH) is a severe inflammatory condition caused by overactive immune cells, often linked to infections, cancers, or autoimmune diseases like systemic lupus erythematosus (SLE).
- A 29-year-old woman, previously healthy, was diagnosed with HLH after presenting with fever, rash, and low blood cell counts, which led to the discovery of SLE.
- Her treatment included dexamethasone, etoposide, and belimumab, resulting in a full recovery, emphasizing the need to investigate rheumatological disorders in HLH cases regardless of past health status.
Article Abstract
Secondary hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory condition caused by the hyperactivation of macrophages and T-cells, triggered by infection, malignancy, or underlying rheumatological conditions. It rarely presents as a first manifestation of a rheumatological condition. Macrophage activation syndrome (MAS) is secondary HLH associated with underlying hematological conditions. Here, we present a case of a previously healthy 29-year-old female who was admitted with fever, rash, and pancytopenia, found to have HLH, and a workup revealed underlying systemic lupus erythematosus (SLE). She was successfully treated with dexamethasone, etoposide, and belimumab, with complete recovery of her symptoms. This case highlights the importance of a thorough evaluation of rheumatological conditions in all patients with HLH despite their previous medical history and the use of belimumab for SLE.
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Source |
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11325255 | PMC |
| http://dx.doi.org/10.7759/cureus.64596 | DOI Listing |
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