Background
Hemophagocytic lymphohistiocytosis (HLH), commonly known as hemophagocytic syndrome, is a rare and serious disease triggered by a myriad of stimuli. HLH is characterized by an uncontrolled cytokine production and persistent lymphocyte and macrophage activation which results in hemophagocytosis. Since people living with human immunodeficiency virus (PLWH) have an increased risk of coinfections, HLH has great relevance in the clinical field as it worsens patient’s prognosis.
Methods
We conducted a descriptive retrospective study to elucidate the main characteristics of secondary HLH in PLWH. This study included 23 participants with more than 90% probability for HLH by HSCORE, enrolled from three HIV reference centers in Mexico City from January 2019 to December 2023. Clinical and microbiological data were collected from clinical records, as well as bone diagnostic biopsies to perform Epstein–Barr virus–encoded small RNAs (EBER) by in situ hybridization, and to confirm infectious or neoplastic diseases.
Results
From 23 participants, 4 (17%) were cisgender women, 3 (13%) transgender women, and 16 (70%) cisgender men. Median HIV viral load (VL) was 5.48 (IQR=2.69-6.47) log copies/mL, and the median CD4 T cell count was 24.50 (IQR=15-65) cells/mL. Immune reconstitution inflammatory syndrome was presented in 5 (22%) individuals, and 8 (35%) died. Fungal diseases were the most common trigger (n=14 [61%] individuals) which included H. capsulatum or Cryptococcus spp. infections. Viral (n=6 [26%], including influenza and SARS-CoV-2) and mycobacterial infections (n=6 [26%]) were the second most common trigger, followed by neoplastic processes (n=4 [17%]) such as Hodgkin lymphoma and non-Hodgkin lymphoma. CMV and EBV VL were positive in 5 (22%) and 6 (26%) subjects respectively. Multiple coinfections were presented in 16 (70%) participants with 11 (48%) having two coinfections and 5 (22%) having more than two. In bone biopsies, erythrophagocytosis was confirmed in 9 (39%) individuals. EBER was performed in 4 bone marrow biopsies, all being positive (Figure 1).
Conclusions
HLH confers high risk of mortality. In this study, HLH was presented in a context of high HIV VL, low CD4 count, and multiple coinfections. Since mortality was a common outcome, HLH should be considered in critically ill HIV patients.
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