Abstract
BackgroundHemophagocytic lymphohistiocytosis (HLH) is a rare disorder that can be either primary (genetic) or secondary (reactive) in etiology. Diagnosis can be elusive, especially in patients with HIV infection.MethodsMedical records were reviewed for 5 patients with HIV infection and the diagnosis of HLH. Standard and alternative criteria were utilized to establish the diagnosis.ResultsFive patients with HIV infection had clinical criteria for the diagnosis of HLH. Ages ranged from 33–70 years and 4 were males. All five presented with fevers, cytopenias, and markedly elevated ferritin levels (table). All of the patients had CD4 levels of < 200 cells/µL. Evidence of hemophagocytosis was found on bone marrow examination in 3 patients. Inciting conditions included Pneumocystis jiroveci infection, EBV infection, lymphoma, and multiple myeloma. All patients received broad-spectrum antimicrobial as well as immunosuppressive therapy. Despite aggressive treatments, all patients died within one month of presentation.ConclusionIn patients with underlying HIV infection, HLH can be a difficult diagnosis to establish. Mortality rates can be high, even with prompt recognition and therapy. The finding of fever and cytopenia in a patient with HIV infection should prompt the clinician to determine a ferritin level. If markedly elevated, the diagnosis of HLH should be aggressively pursued. Disclosures All authors: No reported disclosures.
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