Slide Session : OS-HEM-03 ; Hematology : Hemophagocytic Syndrome in Internal Medicine: Iden-tification of Infectious Triggers, Therapeutic Manage-ment and Mortality in 88 Patients (REGHEM-GEAS-SEMI) = Slide Session : OS-HEM-03 ; Hematology : Hemophagocytic Syndrome in Internal Medicine: Iden-tification of Infectious Triggers, Therapeutic Manage-ment and Mortality in 88 Patients (REGHEM-GEAS-SEMI)

다국어 초록 (Multilingual Abstract)

Background: To analyze the therapeutic management and the main epidemiological and clinical characteristics related to survival in a large series of patients with hemophagocytic syndrome (HS) diagnosed in Departments of Internal Medicine. Methods: In June 2013, the Study Group of Autoimmune Diseases (GEAS-SEMI) creates a national registry of patients with HS. Patients were diagnosed according to the fulfillment of the criteria of the Histiocytosis Society in 1991 and updated in 2004. Results: At June 15, 2014, the REGHEM registry included 88 patients with HS, 35 (40%) men and 55 (60%) women, with a mean age at diagnosis of 49.16 years (range 12- 84 years). During the admission that led to the diagnosis of SH, acute infections were identified in 50 (57%) patients, including viruses (n=20), bacteria (n=13), mycobacteria (n=8) and parasites/fungi (n=13). Patients were treated with corticosteroids (n=40), etoposide (n=12), cyclosporin A (n=12), methotrexate (n=4), tacrolimus (n=2) rituximab (n=3), intravenous immunoglobulins (n=2) and chemotherapy (n=5). A total of 44 (50%) patients died. The main factors associated with mortality were analytical parameters at diagnosis such as the presence of severe anemia (Hb <9 g/L, 89% vs 66%, p=0.01), platelet count <100,000/mm3 (96% vs 64%, p<0.001), neutropenia <1000/mm3 (59% vs 39%, p=0.044), and bacterial infections as precipitating agent (77% vs 45%, p=0.034); a clinical presentation including lymphadenopathy was associated with a lower mortality (59% vs 39%, p=0.044). Conclusions: Hemophagocytic syndrome is a severe multisystemic disease associated in nearly 50% of cases with an acute infection, and usually requires vital support in intensive care units. Despite this and the use of a complex therapeutic approach, half of the patients died. The main prognostic factor identified is the presence at diagnosis of severe cytopenias and the coexistence of bacterial infections.