title: Comorbidities Drive Outcomes for Both Malignancy-Associated and Non-Malignancy-Associated Hemophagocytic Syndrome.
abstract: BACKGROUND
  Secondary hemophagocytic syndrome (SHPS) is a syndrome that develops as a result of infection, autoimmunity, or underlying malignancy. We studied novel predictors of mortality among adults with SHPS.
  
  
  METHODS
  SHPS were identified from the Nationwide Inpatient Sample for 2009 to 2011 using International Classification of Diseases, 9th Revision, Clinical Modification (ICD-9-CM), codes. Charlson comorbidity index (CCI) was used for comorbidity assessment, excluding malignancy. Patient- and hospital-related factors on mortality were assessed by chi-square test or analysis of variance. P values were 2 sided, and the level of significance was .05.
  
  
  RESULTS
  A total of 276 patient hospitalizations with SHPS were identified. Forty-four had an associated malignancy, 38 (86%) of which were hematologic. Median age was 42 years (range, 18-89 years). A total of 66% (n = 182) had a CCI of 0, 13% (n = 27) had a CCI of 1, and 21% (n = 57) had a CCI of 2 or more. On bivariate analysis, inpatient mortality rate was significantly higher in malignancy-associated hemophagocytic syndrome (HPS) (odds ratio [OR], 2.07; P = .04), age ≥ 50 years (OR, 3.46; P < .01), CCI ≥ 2 (OR, 3.04; P < .01), and Medicare patients (OR, 2.32; P < .01). In multivariate analysis, CCI ≥ 2 remained an independent predictor of survival in the overall study cohort (OR, 3.52; 95% confidence interval, 1.51-8.18; P < .01).
  
  
  CONCLUSIONS
  Malignancy-associated HPS, CCI ≥ 2, age > 50 years, and Medicare patients were associated with a worse in-hospital mortality. In multivariate analysis, greater comorbidity burden appeared to be the single most important predictor of mortality. This suggests that outcomes for adults with HPS are predicated by the extent of organ dysfunction at diagnosis.
authors.affiliation: Division of Hematology/Oncology, Department of Medicine, The University of Tennessee Health Science Center, Memphis, TN. Electronic address: bejohnson@westclinic.com.
authors.forename: Benny
authors.initials: B
authors.lastname: Johnson
authors.affiliation: Department of Medicine, The University of Tennessee Health Science Center, Memphis, TN.
authors.forename: Smith
authors.initials: S
authors.lastname: Giri
authors.affiliation: Department of Medicine, The University of Tennessee Health Science Center, Memphis, TN.
authors.forename: Sara E
authors.initials: SE
authors.lastname: Nunnery
authors.affiliation: Division of Hematology/Oncology, Department of Medicine, The University of Tennessee Health Science Center, Memphis, TN.
authors.forename: Eric
authors.initials: E
authors.lastname: Wiedower
authors.affiliation: Department of Medicine, The University of Tennessee Health Science Center, Memphis, TN.
authors.forename: Omer
authors.initials: O
authors.lastname: Jamy
authors.affiliation: Division of Hematology/Oncology, Department of Medicine, The University of Tennessee Health Science Center, Memphis, TN.
authors.forename: George
authors.initials: G
authors.lastname: Yaghmour
authors.affiliation: Department of Hematology/Oncology, The West Cancer Center, The University of Tennessee Health Science Center, Memphis, TN.
authors.forename: Jason C
authors.initials: JC
authors.lastname: Chandler
authors.affiliation: Department of Hematology/Oncology, The West Cancer Center, The University of Tennessee Health Science Center, Memphis, TN.
authors.forename: Mike G
authors.initials: MG
authors.lastname: Martin
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015897:Comorbidity; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications*; D008875:Middle Aged; D055815:Young Adult
keywords: Autoimmune disease; Charlson comorbidity index; Malignancy; Organ dysfunction; Secondary hemophagocytic syndrome