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

TL;DR

A case report describes a transgender woman with HIV who developed fatal hemophagocytic lymphohistiocytosis triggered by extrapulmonary tuberculosis.

Contribution

Highlights the diagnostic challenge of HLH in HIV patients and the importance of rapid TB detection using LF-LAM tests.

Findings

HLH in an HIV patient was triggered by extrapulmonary tuberculosis, confirmed via LF-LAM and bone marrow biopsy.

Despite combined immunomodulation and antituberculosis therapy, the patient's prognosis remained poor due to advanced immunosuppression.

The case underscores the need for high clinical suspicion in HIV-positive individuals with fever and cytopenias in TB-endemic regions.

Abstract

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.

Figures