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- 저자 : ( Pilar BRITO ZERÓN ) (검색결과 2 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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( Pilar BRITO ZERÓN ),( Marta PEREZ DE LIS NOVO ),( Belchin KOSTOV ),( Roser SOLANS ),( Guadalupe FRAILE ),( Carlos SUÁREZ CUERVO ),( Arnau CASANOVAS ),( Francisco Javier RASCÓN ),( Rami QANNETA ),( R 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1
Background: To analyze prognostic factors and standardized mortality ratio (SMR) with respect to the general population in a cohort of Spanish patients with primary Sjogren`s syndrome (SS). Methods: In October 2013, the RESSP-GEAS-SEMI database included 1045 consecutive patients who met the 2002 SS-criteria. Hazard ratios (HR) and confi dence intervals (95% CI) obtained in the adjusted regression model were calculated. The SMR was estimated using the life tables of the general population of Spain in 2012. Results: The cohort included 982 (94%) women with a mean age at diagnosis of 54 years and a mean disease evolution of 118 months; 115 (11%) patients died due to systemic disease (n=18), infection (n=21), cardiovascular disease (n=35), hematologic malignancy (n=10) and other causes (n=31). The SMR for the total cohort of patients (adjusted for age and sex with the general Spanish population) was 4.66. Survival rates at 5, 10, 20 and 30 years were 96.0%, 90.5%, 80.9% and 60.4%, respectively. The Cox-regression analysis identifi ed the following baseline variables at diagnosis associated with death: male gender (HR 2.98, p<0.001), altered parotid scintigraphy (HR 2.81, p=0.043), lymphopenia (HR 1.63, p=0.034), anti-La antibodies (HR 1.51, p=0.034), low C3 (HR 1.93, p=0.034), low C4 (HR 2.06, p=0.016), monoclonal gammopathy (HR 1.81, p=0.047) and cryoglobulins (HR 2.58, p<0.001). The main baseline factors associated with mortality caused by systemic disease were systemic-activity at diagnosis, cytopenias, monoclonal gammopathy, cryoglobulins, and hypocomplementemia. Conclusions: Primary SS should not be considered a mild disease, since mortality is almost 5 times greater with respect to general population, with an overall survival at 20 years of 81%. Patients with a lower survival are those who present with active disease at diagnosis and associated immunological markers of B-cell hyperactivity.
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