title: [Hemophagocytic lymphohistiocytosis: Analysis of 18 cases].
abstract: OBJECTIVE
  Hemophagocytic lymphohistiocytosis (HLH) is a serious condition, caused by an improper regulation of the immune response to different stimuli of the immune system. Early diagnosis and treatment are a challenge for the clinician.
  
  
  METHODS
  We conducted a retrospective study at our institution between 2010 and 2015, of adult patients diagnosed with HLH, in accordance with the criteria of the Histiocyte Society, analyzing their clinical characteristics, diagnostic and etiological studies and the outcome.
  
  
  RESULTS
  Eighteen patients were analyzed. Median time to diagnosis was 24 days. We found neoplastic etiology in 8 cases (7 hematologic), while it was infection-related in 6 (4 visceral leishmaniasis), and an inflammatory disease in one. In the remaining 3, an underlying cause for the HLH was not found. Course of treatment was corticosteroids in 16 patients, associated with cyclosporine in 2 of them, one received immunoglobulins, while another received etoposide with tacrolimus.
  
  
  CONCLUSIONS
  We emphasize the scarce use of etoposide therapy, the currently recommended treatment. Overall mortality was 44%, mainly associated with neoplastic etiology (67 compared to 16.6% mortality in infection-related etiology, P<.05).
authors.affiliation: Servicio de Medicina Interna, Hospital Universitario 12 de Octubre, Madrid, España. Electronic address: pilihj@hotmail.com.
authors.forename: Pilar
authors.initials: P
authors.lastname: Hernández-Jiménez
authors.affiliation: Servicio de Medicina Interna, Hospital Universitario 12 de Octubre, Madrid, España.
authors.forename: Carmen
authors.initials: C
authors.lastname: Díaz-Pedroche
authors.affiliation: Servicio de Medicina Interna, Hospital Universitario 12 de Octubre, Madrid, España.
authors.forename: Jaime
authors.initials: J
authors.lastname: Laureiro
authors.affiliation: Servicio de Medicina Interna, Hospital Universitario 12 de Octubre, Madrid, España.
authors.forename: Olaya
authors.initials: O
authors.lastname: Madrid
authors.affiliation: Servicio de Hematología, Hospital Universitario 12 de Octubre, Madrid, España.
authors.forename: Estela
authors.initials: E
authors.lastname: Martín
authors.affiliation: Servicio de Medicina Interna, Hospital Universitario 12 de Octubre, Madrid, España.
authors.forename: Carlos
authors.initials: C
authors.lastname: Lumbreras
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012189:Retrospective Studies; D013030:Spain / Q000453:epidemiology; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000893:Anti-Inflammatory Agents; D007155:Immunologic Factors
keywords: Adult; Adulto; Clinical presentation; Etiology; Etiología; Hemophagocytic syndrome; Macrophage activation syndrome; Outcome; Presentación clínica; Pronóstico; Síndrome de activación macrofágica; Síndrome hemofagocítico