THU0542 Is there Any Difference Between Autoimmune or Hemato-Oncology Etiology to Guide us in the Diagnosis of Secondary Macrophage Activation Syndrome?
BackgroundSecondary 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.ObjectivesTo describe and compare demographics, clinical and l...
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Veröffentlicht in: | Annals of the rheumatic diseases 2015-06, Vol.74 (Suppl 2), p.396 |
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Zusammenfassung: | BackgroundSecondary 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.ObjectivesTo 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.MethodsA 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.ResultsNineteen 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 Still's 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.Table 1All patientsAI diseaseOH diseasep between AI and HOAge (years)56 (32)37.5 (26)66 (15)Female Sex10 (52.6%)4 (66.7%)4 (66.7%)0.205Hospitalization Days from admission to diagnosis38 (69)37.5 (46)61 (59)0.216Days from diagnosis until death or end of study430 (1166)1125 (554)67.5 (410)0.07Organomegaly15 (78.9%)5 (83.3%)7 (77.8%)0.792Hemoglobin (mg/dL)7.3 (1.6)7.4 (2.5)6.4 (1.1)0.0866Platelets (/μL)8000 (15000)11000 (66000)2000 (15000)0.0771Leukocytes (/μL)1010 (2990)2840 (1850)100 (590)0.0771Neutrophils (/μL)360 (1770)1068 (1120)0 (20)0.0074Triglycerides (mg/dL)382 (217)414.5 (307)341 (157)0.44Fibrinogen |
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ISSN: | 0003-4967 1468-2060 |
DOI: | 10.1136/annrheumdis-2015-eular.3006 |