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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

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 Fisher's 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 Still's 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. Table 1 Total AI OH Inf. Onc. p (4 group) p (AI and HO) Hospital stay 38 (26–80.5) 38 (37–56) 61 (38–100) 24 (20.5–27.5) 30 0.332 0.548 ICU admission 5 2 4 0 0 Fever 13 5 5 2 1 Organomegaly 12 5 5 2 0 Hemoglobin (mg/dl) 7.4 (6.8–8.2) 7.5 (7.3–8.9) 7 (6.6–7.4) 7.6 (7.4–7.8) 8.6 0.307 0.222 Platelets (/μl) 10000 (5300–19500) 15000 (8000–72000) 16000 (4000–17000) 9500 (9250–9750) 5000 0.618 0.548 Leukocytes (/μl) 1200 (265–2715) 2340 (1660–3340) 50 (10–820) 2025 (1530–2570) 480 0.054 0.016 Neutrophils (/μl) 580 (0–1565) 1006 (650–1130) 0 (0–360) 1160 (580–1740) 230 0.292 0.095 Triglycerides (mg/dl) 412 (264–516) 417 (412–561) 382 (340–427) 373 (323–422) 149 0.416 0.69 Fibrinogen (mg/dl) 212 (103–400) 158 (97–287) 287 (195–478.5) 214 117 0.746 0.4 Ferritin (μg/L) 15300 (2868–16796) 15300 (2422–15359) 16751 (13246–26021) 10347 (10051–10642) 0 0.499 0.413 ALT (mg/dl) 199 (70–465) 512 (395–1080) 199 (175–277) 79 (51–106) 54 0.167 0.222 AST (mg/dl) 199 (96–475) 566 (199–596) 147 (108–270) 133 (83–184) 88 0.36 0....