- HLH can rarely present as the initial manifestation of acute HIV-1 infection, even in previously healthy young adults.
- Diagnosis was supported by pancytopenia, hyperferritinaemia, hypertriglyceridaemia, elevated liver enzymes, bone marrow haemophagocytosis and meeting five HLH-2004 criteria.
- Prompt antiretroviral therapy combined with corticosteroids led to rapid clinical and laboratory improvement, underscoring importance of early recognition and treatment.
Cureus. 2026 Aug 10;18(8):e114263. doi: 10.7759/cureus.114263. eCollection 2026 Aug.
ABSTRACT
Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe disorder of immune dysregulation associated with overwhelming systemic inflammation and multiorgan dysfunction. Infectious diseases are among the most common triggers of secondary HLH, with viral infections predominating. HLH is a recognized complication of advanced HIV infection; however, it remains exceptionally rare during acute HIV infection. We report the case of a previously healthy 19-year-old man admitted with a two-week history of fever, asthenia, myalgia, anorexia, and significant weight loss. Physical examination revealed pallor and cervical lymphadenopathy. Laboratory investigations showed pancytopenia, marked hyperferritinemia, hypertriglyceridemia, elevated liver enzymes, and an increased lactate dehydrogenase level. Bone marrow examination demonstrated hemophagocytosis. The overall findings were consistent with HLH, as five of the eight HLH-2004 diagnostic criteria were met. An extensive workup for secondary HLH triggers, including infectious, autoimmune, and malignant causes, identified acute HIV-1 infection with high-level viremia and CD4 T-cell lymphopenia. The diagnosis of HLH secondary to acute HIV-1 infection was established, and the patient was promptly started on antiretroviral therapy and corticosteroids, resulting in favorable clinical and laboratory outcomes. Although rare, HLH may present as the initial manifestation of acute HIV infection. Early recognition of this association is crucial, as prompt diagnosis and treatment of the underlying HIV infection can significantly improve outcomes.
PMID:42719281 | PMC:PMC13556281 | DOI:10.7759/cureus.114263
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