Multiple infection is independently related to death in adult patients with hemophagocytic syndrome: etiopathogenically-driven multivariate analysis in 151 patients

BRITO ZERON PILAR; MARTÍNEZ-ZAPICO A.; PÉREZ-GUERRERO PATRICIA; VAQUERO-HERRERO MP; MORCILLO CESAR; RUIZ-MUÑOZ M ; ALJIBE AGUILAR A; RODRÍGUEZ FERNÁNDEZ C.; FLORES-CHAVEZ ALEJANDRA; RETAMOZO SOLEDAD; MORAL MORAL PEDRO; FRAILE G; ROBLES ANGEL; M.J. FORNER ; RODRIGUEZ-CARBALLEIRA M; PRIETO-GONZÁLEZ SERGIO; HERNÁNDEZ-JIMÉNEZ P; MORELL MASSÓ V.; KOSTOV, BELCHIN; DÍAZ-PEDROCHE C.; FONSECA EVA; ANDRÉS CALVO M; LARRAÑAGA J; HURTADO-GARCÍA ROBERTO; LUIS CAMINAL-MONTERO ; CASTRO P.; RAMOS-CASALS, MANUEL

Amsterdam

Congreso; EUROPEAN LEAGUE AGAINST RHEUMATISM (EULAR); 2018

EULAR

Objective. To characterize the aetiologies and clinical features at diagnosis of patients with hemophagocytic lymphohistiocytosis (HLH) and correlate these baseline features with survival using an etiopathogenically-guided multivariate model.Methods. The HLH Study Group of the Spanish Group of Autoimmune Diseases (GEAS) was formed in 2013 with the aim of collecting a large series of Spanish adult patients with HLH with substantial experience in the management of patients with systemic diseases. By October 2017, the database included 151 consecutive patients who fulfilled at least 5 of the 8 criteria proposed by the Histiocytosis Society in 2004.Results: The cohort consisted of 151 patients (91 male, mean age 51.4 years). After a mean follow-up of 17 months, 80 (53%) patients died. The HLH-independent variables associated with death were: age (HR 1.03, p 1 infectious trigger (HR 2.95, p=0.003) were associated with death. Of the therapeutic interventions, ID/IVIG/biological therapies (HR 0.44, p=0.039) were significantly associated with mortality. Time-to-event analyses for death identified a worse survival curve for patients with neoplasia (p 1 (p=0.011) infections and glucocorticoid monotherapy (p=0.021). After adjusting for confounding variables, platelets < 100,000/mm3 (HR 2.64), severe hyponatremia (HR 1.88), >1 infectious trigger (HR 3.43) and mixed microbiological infection (HR 2.96) remained significant. Multivariate Cox proportional hazards regression analysis identified >1 infectious trigger (HR 2.60, 95%CI 1.16 to 5.84) as the only variable independently associated with death. Conclusions. The mortality rate of adult patients diagnosed with HLH exceeds 50%. Infection with >1 microbiological agent was the only independent variable associated with mortality irrespective of the underlying disease, epidemiological profile, clinical presentation and therapeutic management.