Infection is the major trigger of adult hemophagocytic syndrome, an orphan systemic hyperinflammatory, life-threatening disease: analysis in 147 patients (GEAS-SEMI Registry)

MORAL MORAL P; FRAILE, GUADALUPE; ROBLES ANGEL; FORNER MJ; VIEJO-LORENTE LF; MORCILLO CÉSAR; BRITO ZERON PILAR; MARTÍNEZ-ZAPICO A; PÉREZ-GUERRERO PATRICIA; VAQUERO-HERRERO M; LARRAÑAGA J; RUIZ-MUÑOZ MANUEL; RETAMOZO SOLEDAD; FLORES-CHAVEZ ALEJANDRA; HERNÁNDEZ-JIMENEZ PILAR; FONSECA, EVA; RUIZ-DE-TEMIÑO A; RODRIGUEZ-CARBALLEIRA M; HURTADO-GARCÍA ROBERTO; RAMOS CASALS MANUEL

San Diego

Congreso; ACR / ARHP ANNUAL MEETING; 2017

American College of Rheumatology

Objective. Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH) is an increasingly-recognized condition in adults, characterized by a wide range of etiologies, symptoms and outcomes, but with a common etiopathogenic pathway leading to organ damage: an excessive hyperinflammatory response. We analyzed the role of infections as triggers of adult hemophagocytic syndrome in a National Cohort Registry.Methods. In June 2013, the Study Group on Autoimmune Diseases (GEAS) of the Spanish Society of Internal Medicine created the national registry of patients with HLH. Patients fulfilled the diagnostic guidelines for HLH proposed by the Histiocyte Society in 1991 and updated in 2004. We investigated infections (viruses, bacteria, parasites and fungi) as causative external factors potentially involved in initiate the disease.Results: On June 15, 2017, a total of 147 patients with HLH were included in the GEAS-SEMI Registry, 87 (59%) men and 60 (41%) women, with a mean age at diagnosis of HLH of 44; 18% were not born in Spain. The main signs and symptoms at presentation consisted of fever in 132 (90%) patients, splenomegaly in 80 (54%), polyadenopathies in 71 (48%) and hepatomegaly in 22 (15%) patients. The main internal organ involvements included pulmonary in 49 (33%) patients, renal in 46 (31%), gastrointestinal in 41 (28%) and CNS in 32 (22%) patients. The main analytical markers consisted of ferritin > 500 ng/mL in 143 (97%, mean 15498 ng/mL), thrombocytopenia