Hemophagocytic Lymphohistiocytosis Secondary to Extrapulmonary Tuberculosis in an HIV Patient: A Case Report.

Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome caused by unregulated immune activation, leading to a "cytokine storm" and multisystem failure. In people living with HIV (PLWH), it is frequently triggered by opportunistic infections like Mycobacterium tuberculosis. This report describes a 28-year-old transgender woman with HIV (on ART, undetectable viral load) admitted with a 20-day history of high fever, diarrhea, and hepatosplenomegaly. Admission labs showed severe pancytopenia (Hb 6.2 g/dL, WBC 1,590/µL, platelets 102,000/µL) and a CD4 count of 164 cells/mm³. Investigation revealed extreme hyperferritinemia (peaking at 18,366 ng/mL) and a positive urinary lateral flow urine lipoarabinomannan assay (LF-LAM) test, indicating extrapulmonary tuberculosis (TB). Abdominal CT showed mesenteric and para-aortic lymphadenopathy. Bone marrow biopsy confirmed HLH through frequent hemophagocytosis figures. Despite immediate initiation of antituberculosis therapy (ATT) combined with intravenous immunoglobulin, methylprednisolone pulse, and cyclosporine, the patient developed acute kidney injury and severe hepatitis, progressing to refractory shock and death on the 37th day. This case highlights the diagnostic challenge of HLH due to clinical overlap with sepsis. The LF-LAM test was crucial for rapid TB identification. Although integrated immunomodulation and ATT are recommended, the prognosis remains poor in advanced immunosuppression. High clinical suspicion is vital for early intervention in HIV-positive patients with fever and cytopenias in TB-endemic regions.