title: Immune response of thalassemia major patients in Indonesia with and without splenectomy.
abstract: OBJECTIVE
  to describe non-spesific and specific immune response profile in Indonesian thalassemia major with and without splenectomy.
  
  
  METHODS
  this study was held at Thalassaemia Centre, Cipto Mangunkusumo Hospital Jakarta on September 2013-February 2014. A comparative cross sectional study was conducted in healthy, thalassemia major aged more than 12 year and seronegative HIV. They were matched in age and sex for splenectomised and non-splenectomised groups, analysing the non-spesific immune response (neutrophil count and phagocytosis) and specific immune response (count and function of cellular immunity). Infection episodes were also analized as immune response in vivo parameter.
  
  
  RESULTS
  splenectomised thalassemia major showed increased neutrophil count but significantly decreased non-spesific immune response (neutrophil phagocytosis). Spesific immune response of splenectomised group presented significantly higher absolute lymphocyte, lymphocyte T, CD4+ and CD8+ counts compared to non-splenectomised thalassemia major (p<0.05). Ratio CD4+/CD8+ were similar in these groups. Serum marker of activated cellular imunity function (IL-2 and TNF-) were similar among two groups. Mild infection episodes on splenectomised and non-splenectomised group were 2.02 (ranged 0 to 12) times and 0.81 (ranged 0 to 8) times (p=0.004), respectively. Severe infection on splenectomised group were sepsis for 2 weeks and diarrhea for 1 week, whereas on non-splenectomised group was typhoid fever for 4 days.
  
  
  CONCLUSIONS
  there were significant differences on immune response among thalassemia major patients. Splenectomised thalassemia major showed a greater degree of susceptibility to infections than non-splenectomised thalassemia major.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Universitas Indonesia - Cipto Mangunkusumo Hospital, Jakarta, Indonesia.
authors.forename: Teny T
authors.initials: TT
authors.lastname: Sari
authors.forename: Djajadiman
authors.initials: D
authors.lastname: Gatot
authors.forename: Arwin A P
authors.initials: AA
authors.lastname: Akib
authors.forename: Saptawati
authors.initials: S
authors.lastname: Bardosono
authors.forename: Sri R S
authors.initials: SR
authors.lastname: Hadinegoro
authors.forename: Alida R
authors.initials: AR
authors.lastname: Harahap
authors.forename: Ponpon S
authors.initials: PS
authors.lastname: Idjradinata
mesh_terms: D000293:Adolescent; D000328:Adult; D015415:Biomarkers / Q000097:blood; D018791:CD4 Lymphocyte Count; D002648:Child; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D007111:Immunity, Cellular; D007214:Indonesia; D007376:Interleukin-2 / Q000097:blood; D008297:Male; D009504:Neutrophils / Q000378:metabolism; D010587:Phagocytosis; D013156:Splenectomy; D014409:Tumor Necrosis Factor-alpha / Q000097:blood; D055815:Young Adult; D017086:beta-Thalassemia / Q000097:blood / Q000276:immunology* / Q000601:surgery
chemical_list: D015415:Biomarkers; D007376:Interleukin-2; D014409:Tumor Necrosis Factor-alpha