title: Reactive hemophagocytic syndrome in adult systemic disease: report of twenty-six cases and literature review.
abstract: OBJECTIVE
  To analyze specific clinical findings, underlying disorders, treatments, outcomes, and prognostic factors for reactive hemophagocytic syndrome (RHS) in systemic disease.
  
  
  METHODS
  Data were collected using standardized forms as part of a French national survey. Adult cases without an underlying malignancy, diagnosed on bone marrow or lymph node biopsy, were included.
  
  
  RESULTS
  Twenty-six cases (7 men, 19 women, mean age 47.4 +/- 17.7 years) were studied. Systemic diseases included systemic lupus erythematosus (n = 14), rheumatoid arthritis (n = 2), adult onset systemic Still's disease (n = 4), polyarteritis nodosa (n = 2), mixed connective tissue disease (n = 1), pulmonary sarcoidosis (n = 1), systemic sclerosis (n = 1), and Sjögren's syndrome (n = 1). RHS occurred in 2 distinct clinical settings in the course of systemic disease. RHS was associated with an active infection in 15 patients (bacterial infections, 10 cases; viral, 3 cases; tuberculosis, 1 case; and aspergillosis, 1 case) and with the onset of a systemic disease alone in 9 cases. Isolated RHS occurred in 2 cases. The overall mortality rate was 38.5%. Two factors were associated with mortality: corticosteroid treatment at the time of RHS diagnosis, and thrombocytopenia (odds ratio = 28, 95% confidence interval = 13.3-238.9).
  
  
  CONCLUSIONS
  When RHS occurs in the course of an active systemic disease (situation only reported in cases of systemic lupus or adult Still's disease), immunosuppressive therapy should be used. In contrast, when RHS is present concomitantly with an active infection, immunosuppressive therapy needs to be lowered and antibiotic therapy should be instituted.
authors.affiliation: Hôpital Cochin, Paris, France. robin.dhote@cch.ap-hop-paris.fr
authors.forename: Robin
authors.initials: R
authors.lastname: Dhote
authors.forename: Jeanne
authors.initials: J
authors.lastname: Simon
authors.forename: Thomas
authors.initials: T
authors.lastname: Papo
authors.forename: Bruno
authors.initials: B
authors.lastname: Detournay
authors.forename: Laurent
authors.initials: L
authors.lastname: Sailler
authors.forename: Marie-Helene
authors.initials: MH
authors.lastname: Andre
authors.forename: Jean-Louis
authors.initials: JL
authors.lastname: Dupond
authors.forename: Claire
authors.initials: C
authors.lastname: Larroche
authors.forename: Anne-Marie
authors.initials: AM
authors.lastname: Piette
authors.forename: Didier
authors.initials: D
authors.lastname: Mechenstock
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Ziza
authors.forename: Jean
authors.initials: J
authors.lastname: Arlaud
authors.forename: Anne-Sophie
authors.initials: AS
authors.lastname: Labussiere
authors.forename: Agnes
authors.initials: A
authors.lastname: Desvaux
authors.forename: Vincent
authors.initials: V
authors.lastname: Baty
authors.forename: Philippe
authors.initials: P
authors.lastname: Blanche
authors.forename: Annette
authors.initials: A
authors.lastname: Schaeffer
authors.forename: Jean-Charles
authors.initials: JC
authors.lastname: Piette
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
authors.forename: Alain
authors.initials: A
authors.lastname: Boissonnas
authors.forename: Boyan
authors.initials: B
authors.lastname: Christoforov
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D000998:Antiviral Agents / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D003520:Cyclophosphamide / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D015616:Histiocytosis, Non-Langerhans-Cell / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011795:Surveys and Questionnaires; D016896:Treatment Outcome
chemical_list: D000900:Anti-Bacterial Agents; D000998:Antiviral Agents; D005938:Glucocorticoids; D003520:Cyclophosphamide