title: Erythropoiesis versus inflammation in Hereditary Spherocytosis clinical outcome.
abstract: OBJECTIVE
  This study aimed to evaluate the relationship between erythropoiesis and inflammation, in Hereditary Spherocytosis (HS) clinical outcome.
  
  
  METHODS
  We studied 26 controls and 82 HS patients presenting mild (n = 49) and severer (n = 33) HS forms. We evaluated plasma levels of EPO, sTfR, ferritin, iron, folic acid, vitamin B12, TNF-α, IFN-γ, elastase and lactoferrin; leukocyte and reticulocyte counts and RPI were determined.
  
  
  RESULTS
  All HS patients showed significantly higher EPO, sTfR, reticulocytes and RPI but only mild HS presented normal hemoglobin levels; the positive significant correlations between EPO and sTfR, reticulocytes and RPI observed in mild HS were not observed in severer HS patients. HS patients presented with higher levels of neutrophils, TNF-α, IFN-γ, elastase, lactoferrin and ferritin.
  
  
  CONCLUSIONS
  Our data show HS as a disease linked to enhanced erythropoiesis that is disturbed in the more severe forms, to which inflammation may contribute, at least in part.
authors.affiliation: Faculdade de Farmácia, Universidade do Porto, Rua Aníbal Cunha, 164, 4050-047, Porto, Portugal; Instituto de Biologia Molecular e Celular (IBMC), Universidade do Porto, Rua do Campo Alegre, 823, 4150-180, Porto, Portugal. Electronic address: srocha@ff.up.pt.
authors.forename: Susana
authors.initials: S
authors.lastname: Rocha
authors.affiliation: Instituto de Biologia Molecular e Celular (IBMC), Universidade do Porto, Rua do Campo Alegre, 823, 4150-180, Porto, Portugal; Instituto de Ciências da Saúde, Universidade Católica Portuguesa, Campus da Asprela, Rua Dr. António Bernardino Almeida, 4200-072, Porto, Portugal. Electronic address: elisio_costa@hotmail.com.
authors.forename: Elísio
authors.initials: E
authors.lastname: Costa
authors.affiliation: Instituto de Biologia Molecular e Celular (IBMC), Universidade do Porto, Rua do Campo Alegre, 823, 4150-180, Porto, Portugal; Centro de Investigação em Ciências da Saúde (CICS), Universidade da Beira Interior, Av. Infante D. Henrique, 6201-506, Covilhã, Portugal. Electronic address: petrorp@ciunix.ubi.pt.
authors.forename: Petronila
authors.initials: P
authors.lastname: Rocha-Pereira
authors.affiliation: Serviço de Hematologia Clínica, Hospital S. João, Alameda Prof. Hernâni Monteiro, 4200-319, Porto, Portugal. Electronic address: mfatfer@hotmail.com.
authors.forename: Fátima
authors.initials: F
authors.lastname: Ferreira
authors.affiliation: Sector de Hematologia Pediátrica, Centro Hospitalar do Porto (CHP) - Hospital Santo António, Largo Prof. Abel Salazar, 4099-001, Porto, Portugal. Electronic address: esmeraldacleto.pediatria@chporto.min-saude.pt.
authors.forename: Esmeralda
authors.initials: E
authors.lastname: Cleto
authors.affiliation: Serviço de Hematologia, Centro Hospitalar do Porto (CHP) - Hospital Maria Pia, Largo Prof. Abel Salazar, 4099-001, Porto, Portugal. Electronic address: hematologia@hmariapia.min-saude.pt.
authors.forename: José
authors.initials: J
authors.lastname: Barbot
authors.affiliation: Instituto de Biologia Molecular e Celular (IBMC), Universidade do Porto, Rua do Campo Alegre, 823, 4150-180, Porto, Portugal; Instituto de Ciências Biomédicas Abel Salazar (ICBAS), Universidade do Porto, Largo Prof. Abel Salazar, 2, 4099-003, Porto, Portugal. Electronic address: alexq@ibmc.up.pt.
authors.forename: Alexandre
authors.initials: A
authors.lastname: Quintanilha
authors.affiliation: Faculdade de Farmácia, Universidade do Porto, Rua Aníbal Cunha, 164, 4050-047, Porto, Portugal; Instituto de Biologia Molecular e Celular (IBMC), Universidade do Porto, Rua do Campo Alegre, 823, 4150-180, Porto, Portugal. Electronic address: luisbelo@ff.up.pt.
authors.forename: Luís
authors.initials: L
authors.lastname: Belo
authors.affiliation: Faculdade de Farmácia, Universidade do Porto, Rua Aníbal Cunha, 164, 4050-047, Porto, Portugal; Instituto de Biologia Molecular e Celular (IBMC), Universidade do Porto, Rua do Campo Alegre, 823, 4150-180, Porto, Portugal. Electronic address: assilva@ff.up.pt.
authors.forename: Alice
authors.initials: A
authors.lastname: Santos-Silva
mesh_terms: D000293:Adolescent; D000328:Adult; D015415:Biomarkers / Q000097:blood; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D004920:Erythropoiesis; D005260:Female; D006801:Humans; D007223:Infant; D007249:Inflammation; D008297:Male; D012720:Severity of Illness Index; D013103:Spherocytosis, Hereditary / Q000097:blood* / Q000473:pathology; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D015415:Biomarkers