AB0921 Secondary Macrophage Activation Syndrome: Report of 13 Clinical Cases and the Difference between the 4 Groups of Diseases

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 Neu