Reactive hemophagocytic syndrome in adults: A multicenter retrospective analysis of 162 patients.
Am J Med. 2014 May 14;
Authors: Rivière S, Galicier L, Coppo P, Marzac C, Aumont C, Lambotte O, Fardet L
Abstract
OBJECTIVES: Current knowledge in reactive hemophagocytic syndrome mainly relies on single-centre case series including a relatively small number of patients. We aimed to identify a multicenter large cohort of adult patients with reactive hemophagocytic syndrome and to describe relevant clinical and laboratory features, underlying conditions and outcome.
METHODS: We conducted a multicenter study in three tertiary care centres in France over a 6-year period. The medical files of 312 patients with suspected hemophagocytic syndrome were retrospectively reviewed. Patients were classified with a positive, negative or undetermined diagnosis of hemophagocytic syndrome by experts' consensus.
RESULTS: Among the 312 patients fulfilling our inclusion criteria, 162 patients were classified as positive hemophagocytic syndrome (male: 67%, median age: 48 [35-62] years). Compared to patients without hemophagocytic syndrome, they suffered more frequently from an underlying immunodepression (45% versus 33%, p=0.03) and they exhibited higher temperature, ferritin, triglycerides, AST, birirubin, LDH and CRP and lower haemoglobin, leucocytes, platelets and sodium levels. Noteworthy only 70% of them had hemophagocytosis features on bone marrow aspiration. Haematological malignancies, especially non-Hodgkin lymphomas, were the main trigger of hemophagocytic syndrome, accounting for 56% of cases. The early mortality rate (i.e. within one month after diagnosis) was 20%. Patients with haematological malignancies-associated hemophagocytic syndrome had a poorer early outcome than those with underlying infection.
CONCLUSION: In this large, multicentre study, haematological malignancies are the main disease associated with hemophagocytic syndrome in adults. Early mortality is high and outcome is influenced by the underlying disease.
PMID: 24835040 [PubMed - as supplied by publisher]