Context: Hemophagocytic lymphohistiocytosis is a rare syndrome with a poor prognosis, characterized by an uncontrolled dysregulation of the immune system. The rarity of this disease makes it difficult to obtain large cohorts. In this study, we analyzed the data of 66 patients: the objective was to describe the epidemiological, clinical, biological and therapeutic characteristics and to compare our results with those already published.
Methods: We conducted a retrospective study at the University Hospital of Montpellier from 2015 to 2021. Patients were included when the diagnosis of HLH was mentioned on the hospitalization report and when the HSCORE was higher than 50% (169). Prognostic analyses were performed by comparing the patients who died from HMH to those who didn't.
Results: The mean age the 66 patients included was 49.2 years, 62% were men. The percentage of deaths was 45.9%. Lymphoma was the main etiology, followed by infections, then autoimmune/autoinflammatory diseases. Fever, splenomegaly, hepatomegaly and organ failure were the main clinical manifestations. Pancytopenia was present in 62% of cases. Ferritin, triglycerides, LDH and AST were highly increased. Advanced age, associated lymphoma, and the severity of cytopenias were linked to a poor prognosis.
Discussion: The study of the clinico-biological, epidemiological and survival data of the patients in our cohort allowed us to confirm previously published data but also to discuss some of them.
Keywords: HLH; Hemophagocytic lymphohistiocytosis; LHH; Lymphohistiocytic activation syndrome; Lymphohistiocytose hémophagocytaire; MAS; Macrophagic activation syndrome; SAM; Syndrome d’activation lymphohistiocytaire; Syndrome d’activation macrophagique.
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