Hemophagocytic lymphohistiocytosis associated with disseminated histoplasmosis in a patient with newly diagnosed HIV infection: a case report
TL;DR
The case of a 31-year-old man with newly diagnosed advanced HIV infection, profound immunosuppression, and a high HIV viral load, who presented with prolonged fever, dyspnea, generalized pustular skin lesions, and severe cytopenias, yielded an HScore of 211 points, corresponding to an estimated 93–96% probability of secondary HLH.
Abstract
Disseminated histoplasmosis (DH) associated with hemophagocytic lymphohistiocytosis (HLH) is an uncommon but potentially fatal complication in people living with HIV (PLWH), particularly in those with advanced immunosuppression. We report the case of a 31-year-old man with newly diagnosed advanced HIV infection, profound immunosuppression (CD4+ T-cell count: 6 cells/µL), and a high HIV viral load (773,417 copies/mL), who presented with prolonged fever, dyspnea, generalized pustular skin lesions, and severe cytopenias. Initial investigations revealed diffuse bilateral interstitial infiltrates, hepatomegaly, anemia, thrombocytopenia, and markedly elevated lactate dehydrogenase and liver enzyme levels. Persistent cytopenias, extreme hyperferritinemia, hypertriglyceridemia, and hepatomegaly yielded an HScore of 211 points, corresponding to an estimated 93–96% probability of secondary HLH. Based on the clinical suspicion of disseminated histoplasmosis, liposomal amphotericin B therapy was initiated, and the diagnosis was subsequently confirmed by a positive urinary Histoplasma capsulatum antigen test. The patient experienced marked clinical, hematologic, and biochemical improvement without requiring HLH-directed immunosuppressive therapy. Early recognition of disseminated histoplasmosis and secondary HLH remains challenging because of their overlapping clinical manifestations. Prompt diagnosis and initiation of antifungal therapy may substantially improve outcomes and reduce mortality.