Fulminant and rapidly fatal hemophagocytic lymphohistiocytosis in patients with HIV infection: A report of five cases and a review

Abstract

Hemophagocytic lymphohistiocytosis is a rare entity and diagnosis can be elusive. Commonly recognized inciting causes include autoimmune disorders, malignancies, and a wide variety of infections. With prompt recognition and treatment, survival rates approach 50%. Five cases of reactive hemophagocytic syndrome in patients with underlying HIV infection are reported. All of the patients had CD3/CD4 cell counts of <200 cells/µL. All cases presented with fever and all had marked cytopenias, with platelet counts falling to 10–15,000/µL. Ferritin levels were >5000 ng/mL in all cases. Concomitant inciting conditions included Epstein–Barr virus infection, Pneumocystis jiroveci, multiple myeloma, and Hodgkin’s lymphoma; in one case, no inciting condition was identified. Despite broad-spectrum antimicrobial and immunosuppressive therapy, all five patients died within one month of presentation. The finding of fever and cytopenia in a patient with underlying HIV infection should prompt the clinician to determine a ferritin level. If markedly elevated, an aggressive work-up for hemophagocytic syndrome should be conducted. However, even with prompt recognition, mortality rates may be high in patients with underlying HIV infection.