title: [Hemophagocytic syndrome in a patient with adult-onset Still's disease: diagnostic problems].
abstract: Hemophagocytic syndrome (HPS) is a rare life-threatening condition caused by massive cytokine release from activated macrophages and lymphocytes. The paper depicts the development of HPS in different infections, malignancies, and autoimmune diseases. It describes a clinical case of hemophagocytic syndrome in a 63-year-old female patient with adult-onset Still's disease and high fever accompanied by neutrophil leukocytosis and a drastic left leukocyte count shift, high procalcitonin levels, hepatosplenomegaly, edematous syndrome, and progressive multiple organ dysfunction with the development of disseminated intravascular coagulation and adult respiratory distress syndrome. The diagnosis of HPS was established according to the diagnostic criteria and verified by autopsy: phagocytes in liver and lung tissues.
authors.affiliation: Education and Research Medical Centre, Department for Presidential Affairs of the Russian Federation, Moscow, Russia; V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia.
authors.forename: A Yu
authors.initials: AY
authors.lastname: Zakharova
authors.affiliation: Education and Research Medical Centre, Department for Presidential Affairs of the Russian Federation, Moscow, Russia.
authors.forename: Z Yu
authors.initials: ZY
authors.lastname: Mutovina
authors.affiliation: Education and Research Medical Centre, Department for Presidential Affairs of the Russian Federation, Moscow, Russia; V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia.
authors.forename: A V
authors.initials: AV
authors.lastname: Gordeev
authors.affiliation: Central Clinical Hospital with Polyclinic, Department for Presidential Affairs of the Russian Federation, Moscow, Russia; Peoples' Friendship University of Russia, Moscow, Russia.
authors.forename: I N
authors.initials: IN
authors.lastname: Shestakova
mesh_terms: D017809:Fatal Outcome; D005260:Female; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis* / Q000209:etiology; D008875:Middle Aged; D016706:Still's Disease, Adult-Onset / Q000150:complications*