Hemophagocytic lymphohistiocytosis in HIV-associated lymphoproliferative disorders.

Abstract

Compared tothe general population the incidence of lymphoproliferative disorders (LPDs) is significantly elevated among peopleliving with HIV (PLHIV). In high-income countries LPDs have become the most common HIV-associated cause ofdeath among PLHIV. Lymphomas are one of the most frequent triggers of HemophagocyticLymphohistiocytosis (HLH), a life-threatening inflammatory syndrome that manifests as a sepsis-like syndrome thusobscuring the underlying condition and delaying its diagnosis and therapy. We performed this retrospectivecohort study comprising all adult HIV-infected patients who started treatment for histologically proven LPDsbetween October 2013 and July 2019, to analyse risk factors, frequency and outcome of HLH among HIV-infectedpatients with LPDs. Of 75 patients, six (8%) presented with or developed HLH. Three patients had Hodgkinlymphoma and three had HHV-8 associated diseases. There was a significant correlation (p<0.01) between bonemarrow involvement and the development of HLH. HLH was associated with lower overall survival (HR: 5.09;95%CI: 1.53 - 16.91 p=0.008). In conclusion HLH appears to be more frequent in HIV-associated lymphomasthan in HIV-negative lymphomas. The probability of developing HLH was particularly high in patients with Hodgkinlymphoma, lymphoma with bone marrow infiltration and HHV-8 associated lymphoma. Mortality was significantlyincreased in the presence of HLH.