A Review of Hemophagocytic Lymphohistiocytosis in Patients Living with Human Immunodeficiency Virus

Abstract We provide an elaborate review of cases published between January 2005 and April 2021 on hemophagocytic lymphohistiocytosis (HLH) in HIV patients. Seventy articles describing 81 adult patients (age ≥19 years) were included. Median age was 40 years, and 78% were males. Only 65% were known to have HIV prior to presentation. CD4 count was ≥200 cells/mm3 in 23% and HIV viral load <200 copies/mL in 41%. The lack of meticulous reporting of ≥5 of 8 criteria for HLH diagnosis was evident in a third of cases. At least one infectious agent - other than HIV- was believed to trigger HLH in 78% of patients. The most common were EBV (26%), HHV-8 (21%) and Histoplasma capsulatum (17%). Sixty percent survived. Among those, 93% received treatment for identified secondary trigger(s) while 51% received HLH directed therapy. There was significant heterogeneity in the treatment regimens used for HLH.