title: [Secondary hemophagocytic syndrome due to recurrent infections in a severely burned patient].
abstract: The hemophagocytic syndrome represents an infrequent, occasionally misdiagnosed and usually fatal heterogeneous entity. Infections, drugs, autoimmune diseases and cancer are often triggers of the secondary hemophagocytic syndrome. Its physiopathogenic mechanism is explained by an impaired and inefficacious function of the NK and T cytotoxic cells that leads to an ineffective and uncontrolled immune response, inducing cellular damage, multiorganic failure with macrophage proliferation and hemophagocytosis. The main objective of the different therapeutic options, commonly combinations of steroids and chemotherapy, is the suppression of the uncontrolled immune response. Occasionally, the clinical condition of some patients represents a contraindication for intensive treatment. We report a case of a severely burned patient that fulfilled the revised criteria for the hemophagocytic syndrome and was successfully treated with the combination of intravenous immunoglobulins and steroids.
authors.affiliation: Servicio de Hematología, Hospital Alemán, Buenos Aires, Argentina. cmahuad@hospitalaleman.com
authors.forename: Carolina V
authors.initials: CV
authors.lastname: Mahuad
authors.forename: Gonzalo M
authors.initials: GM
authors.lastname: Garate
authors.forename: María de Los Ángeles
authors.initials: Mde L
authors.lastname: Vicente Reparaz
authors.forename: Claudia
authors.initials: C
authors.lastname: Casali
authors.forename: Mercedes
authors.initials: M
authors.lastname: Del Olmo
authors.forename: Alberto
authors.initials: A
authors.lastname: Bolgiani
mesh_terms: D002056:Burns / Q000150:complications*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage; D051359:Lymphohistiocytosis, Hemophagocytic / Q000188:drug therapy / Q000209:etiology*; D008262:Macrophage Activation; D008297:Male; D012008:Recurrence; D013256:Steroids / Q000008:administration & dosage; D013577:Syndrome; D014946:Wound Infection / Q000188:drug therapy / Q000209:etiology*; D055815:Young Adult
chemical_list: D016756:Immunoglobulins, Intravenous; D013256:Steroids
keywords: hemophagocytic lymphohistiocytosis; hemophagocytosis; intravenous immunoglobulins