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RISS 인기검색어
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- 저자 : ( Angela RUI (검색결과 1 건)
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( Pilar BRITO ZERÓN ),( Marta PEREZ DE LIS NOVO ),( Roberto PÉREZ ALVAREZ ),( Pedro MORAL MORAL ),( Aleida MARTÍNEZ ZAPICO ),( Guadalupe FRAILE ),( Eva FONSECA ),( María VAQUERO HERRERO ),( Angela RUI 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1
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.
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