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Background Secondary macrophage activation syndrome (MAS) is a group of diseases, especially due to autoimmune (AI) and hemato-oncology (HO). It would be interesting to find any clinical and analytics data to differentiate both etiologies. Objectives To describe and compare demographics, clinical and laboratories features and mortality of patients diagnosed with secondary MAS due to HO and AI diseases at the Donostia University Hospital. Methods A cohort of patients diagnosed with MAS was studied by reviewing medical reports in the period Dec/2008-Jan/2015. We analyzed and compared only patients with AI and HO diseases. The variable studied were diagnosis, age, sex, fever, organomegaly, hospital and overall mortality, analytical findings, hospital stay, days from admission to diagnosis and days from diagnosis to the end of the study or death after discharge. Quantitative variables are shown with the median and interquartile range. For the bivariate analysis Wilcoxon and Chi square test were used. Median survival in months and Hazar Ratio (HR) was calculated with the Kaplan Meier plot. Results Nineteen patients were found diagnosed with MAS, 6 and 9 were due to AI and HO diseases respectively. The AI diseases found were: 3 Systemic Lupus Erythematosus, 2 Adult Still9s Disease and 1 disease associated with IgG4. HO diseases found were: 3 acute myeloid leukemias, 2 B cells Not Hogkin Lymphoma (NHL), 1 T anb B cells NHL, 1 Natural Killers cells extranodal lymphoma, 1 myelodysplastic syndrome and 1 gastric plasmacytoma. The table shows the descriptive analysis of 19 patients and the bivariate analysis between AI and HO. The median survival of HO diseases was 1.2 months and AI diseases can not be calculated. The mortality of HO and AI diseases was 77.8% and 16.7% respectively with a p=0.04 in the log rank test. The HR of mortality among HO against AI diseases was 6.84. Conclusions In the present study the following differences were found: 1) Patients with MAS due to AI diseases have a higher elevated liver enzymes compared to HO disease. 2) Patients with MAS due to HO diseases have a greater pancytopenia compared to AI diseases, especially in leukocytes and neutrophils. 3) Patients with MAS due to HO have a higher mortality compared to AI diseases. Disclosure of Interest None declared

Background The secondary macrophage activation syndrome (SMAS) is a group of diseases that include the: Autoimmune (AI), Hemato-Oncological (HO), Infectious (Inf) and Oncological (Onco). We make a review of the the cases presented in a our hospital. Objectives Describe the characteristics demographic, clinical, laboratory, treatment, underlying disorders and mortality of patients diagnosed with SMAS. Methods A cohort was studied retrospectively of patients diagnosed with SMAS between the period December/2008-January/2014 by reviewing medical records from the diagnosis until January/2014. The nominal variables were: diagnosis, treatments during hospitalization and after discharge. Dichotomous variables were: sex, fever, organomegaly, mortality, admission to the Intensive Care Unit (ICU) and recurrence of SAMS. Quantitative variables were: age, laboratory findings, hospital stay, days from admission to the bone marrow biopsy (BMB). Patients were divided into 4 groups (AI, HO, Inf and Onc). The variables were analyzed between the 4 groups and between AI and HO. Quantitative variables showed asymetric distribution so it showed with the median and interquartile range. For the bivariate analysis the Kruskal-Wallis, Pearson Chi Square and Fisher9s Exact tests were used. Results 13 patients (6 female) with median age of 54 (32-63) were found: 5 with AI diseases (2 Systemic Lupus Erythematosus, 2 Adult Still9s Disease and 1 IgG4 associated disease), 5 HO diseases (3 Non Hogkin Lymphoma, 1 acute myeloid leukemia and 1 Lymphoma of Natural Killers cells), 2 Inf. diseases (Campylobacter jejuni and human immunodeficiency virus) and 1 Onc. disease (Chemotherapy in Glioblastoma multiforme). Table shows the descriptive and bivariate analysis of the most important variables. The treatments used were: glucocorticoids in all patients, immunoglobulins in 8 (3 AI, 2 HO, 2 Onc. and 1 Inf.), Cyclosporine in 8 (5 AI and 3 HO), anakinra in 4 (2 HO and 2 AI), tocilizumab and chemotherapy in 2 HO. Conclusions We found that leukopenia was lower in AI disease than HO diseases with statistically significant p. AI diseases had worse prognosis, increased mortality and hospital days than other diseases. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.5055

Background Hemophagocytic Lymphohistiocytosis (HLH) is a severe and acute inflammatory syndrome, underdiagnosed, difficult to treat and can occur at any stage of life. The macrophage activation syndrome is a variant of secondary HLH occurs in autoimmune diseases.1 Objectives Describe the clinical, laboratory, treatment and outcome of patients diagnosed with secondary HLH, identifying probable etiological and triggers causes. Methods A retrospective study was performed between 2008 and 2012 at the Donostia University Hospital, Spain. Inclusion criteria were to met diagnostic criteria for HLH and had a bone marrow biopsy with hemophagocytics cells. Mains endpoints were: demographics, diagnostic criteria, probable etiology, triggers causes and treatments. Secondary endpoints were: time delay in diagnosis, days of hospitalization, need for admission to the Intensive Care Unit (I.C.U.) and the cause, and overall mortality. Results We recruited 11 patients (7 men and 4 women) with a mean age of 48.9 years (16 - 78 years). The below table describes the probable etiologies and triggers of secondary HLH. As no data in the literature described, we found as etiologies and triggers causes of secondary HLH: Campylobacter jejuni in a previously healthy patient without comorbidities; Pneumocystis jirovecii in a patient with Human immunodeficiency virus (H.I.V.); and patient with grade IV glioblastoma multiforme after starting chemotherapy with temozolomide. Hemophagocytics cells were found in ascitic fluid in one patient with Adult Still´s Disease. The mean delay in diagnosis was 14.5 days (3 – 31 days) and average time of hospitalization was 46.7 days (10 – 130 days). The 45% of patient required admission in I.C.U, the leading cause was the multiple organ failure (MOF). The overall mortality rate was 36.4% (4 MOF and 1 massive hemoptysis). Treatment given was steroids, synthetic immunosuppressants and biological drugs. Conclusions The secondary HLH should be suspected in any patient with prolonged fever unresponsive to broad-spectrum antibiotics, hepatosplenomegaly, cytopenias, coagulation and liver disorders. Hemophagocytics cells might be found in pathological body fluids before a bone marrow biopsy had been done or when there are doubts in the result of it. Meet all the diagnostic criteria is not necessary to start treatment when you have a high clinical suspicion and a bone marrow biopsy with hemophagocytics cells. Mortality can be influenced by etiology, trigger cause, and diagnosis and treatment delay. References Henter JI, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007; 48:124. Disclosure of Interest None Declared