title: Reactive haemophagocytic syndrome in 58 HIV-1-infected patients: clinical features, underlying diseases and prognosis.
abstract: OBJECTIVE
  To describe features of reactive haemophagocytic syndrome (RHS) in HIV-1-infected adult patients. To compare characteristics of patients with malignancy-associated RHS and infection-associated RHS.
  
  
  METHODS
  Retrospective study in three departments of Infectious Diseases/Internal Medicine at three French tertiary centres.
  
  
  METHODS
  Medical charts of HIV-1-infected adult patients and RHS seen between January 2006 and December 2007 were reviewed. Demographic, clinical and laboratory data obtained at the time of RHS episode were compared between patients with malignancy-associated RHS and infection-associated RHS using non-parametric tests. The overall survival was assessed using the Kaplan-Meier method.
  
  
  RESULTS
  Fifty-eight HIV-1-infected patients were diagnosed with RHS [certain RHS n = 43, possible RHS n = 15, median (range) age 42 (23-85) years, men 76%]. At time of RHS, the median duration of HIV infection was 4 (0-22) years and 57% received HAART. The median CD4 lymphocyte count was 91 (2-387)/microl and 35% of patients had a plasma HIV-1 RNA less than 50 copies/ml. Underlying haemopathy/malignancy (Hodgkin lymphoma n = 10) or infection (tuberculosis n = 9, cytomegalovirus infection n = 5) were evidenced for 31 and 23 patients, respectively. Patients with haemopathy/malignancy-associated RHS presented more frequently with splenomegaly (97 vs. 70%, P < 0.01), lower aspartate aminotransferase (36 vs. 84 UI/l, P < 0.01) and lactate dehydrogenase (530 vs. 911 UI/l, P < 0.01) levels and CD8 cell count (234 vs. 588/microl, P < 0.01). Eighteen (31%) patients died. The overall survival was not statistically different between the two groups (P = 0.68).
  
  
  CONCLUSIONS
  In the HAART era, RHS is frequently associated with underlying haemopathy/malignancy, especially Hodgkin lymphoma. The prognosis remains poor but seems, however, better than in the pre-HAART era.
authors.affiliation: hôpital Saint-Antoine, France. laurence.fardet@sls.aphp.fr <laurence.fardet@sls.aphp.fr>
authors.forename: Laurence
authors.initials: L
authors.lastname: Fardet
authors.forename: Olivier
authors.initials: O
authors.lastname: Lambotte
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Meynard
authors.forename: Wassim
authors.initials: W
authors.lastname: Kamouh
authors.forename: Lionel
authors.initials: L
authors.lastname: Galicier
authors.forename: Christophe
authors.initials: C
authors.lastname: Marzac
authors.forename: Adrienne
authors.initials: A
authors.lastname: de Labarthe
authors.forename: Jean
authors.initials: J
authors.lastname: Cabane
authors.forename: Céleste
authors.initials: C
authors.lastname: Lebbe
authors.forename: Paul
authors.initials: P
authors.lastname: Coppo
authors.forename: Jean-Michel
authors.initials: JM
authors.lastname: Molina
authors.forename: Valérie
authors.initials: V
authors.lastname: Martinez
mesh_terms: D017088:AIDS-Related Opportunistic Infections / Q000401:mortality / Q000821:virology; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D023241:Antiretroviral Therapy, Highly Active; D018791:CD4 Lymphocyte Count; D005260:Female; D005602:France; D015658:HIV Infections / Q000150:complications* / Q000188:drug therapy / Q000401:mortality; D015497:HIV-1 / Q000276:immunology*; D006801:Humans; D053208:Kaplan-Meier Estimate; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000401:mortality / Q000821:virology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D055815:Young Adult