title: The Fc receptor polymorphisms and expression of neutrophil activation markers in patients with sickle cell disease from Western India.
abstract: OBJECTIVE
  Sickle cell disease has variable clinical manifestations. Activation of neutrophils plays an important role in the initiation and propagation of vaso occlusive crises which can be analysed by determining the expression of neutrophil antigens such as CD16, CD32, and CD62L. The common FcγR polymorphisms (FcγRIIA and FcγRIIIB) are considered to influence clinical presentation. This study focuses on distribution of FcγR polymorphisms and their association with neutrophil activity among the patients from western India.
  
  
  METHODS
  In this paper 127 sickle cell anemia patients and 58 patients with sickle-β-thalassemia (median age 12 ± 8.58 years) with variable clinical phenotypes along with 175 normals were investigated. FcγRs polymorphisms were analysed by RFLP and AS-PCR. Activation of neutrophils was measured by flow cytometry.
  
  
  RESULTS
  The genotypic frequency of the H/R genotype of FcγRIIA and the NA1/NA1 genotype of FcγRIIIB was significantly decreased in patients compared to normals (P-0.0074, P-0.0471, resp.). We found a significant difference in the expression of CD32 and CD62L among the patients as against normals. A significantly higher expression of CD32 was seen in the milder patients with the H/H genotype (P-0.0231), whereas the expression of CD16 was higher in severe patients with the NA2/NA2 genotype (P-0.0312).
  
  
  CONCLUSIONS
  The two FcγR polymorphisms had significant association with variable phenotypes of sickle cell disease. The expression of CD62L decreased in our patients indicating activation of neutrophils.
authors.affiliation: Department of Hematogenetics, National Institute of Immunohaematology (Indian Council of Medical Research), 13th floor, New Multistoried Building, KEM Hospital Campus, Parel, Mumbai 400012, India.
authors.forename: Harshada K
authors.initials: HK
authors.lastname: Kangne
authors.forename: Farah F
authors.initials: FF
authors.lastname: Jijina
authors.forename: Yazdi M
authors.initials: YM
authors.lastname: Italia
authors.forename: Dipti L
authors.initials: DL
authors.lastname: Jain
authors.forename: Anita H
authors.initials: AH
authors.lastname: Nadkarni
authors.forename: Maya
authors.initials: M
authors.lastname: Gupta
authors.forename: Vandana
authors.initials: V
authors.lastname: Pradhan
authors.forename: Rati D
authors.initials: RD
authors.lastname: Mukesh
authors.forename: Kanjaksha K
authors.initials: KK
authors.lastname: Ghosh
authors.forename: Roshan B
authors.initials: RB
authors.lastname: Colah
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000235:genetics* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D058851:GPI-Linked Proteins / Q000096:biosynthesis / Q000235:genetics; D015870:Gene Expression; D006801:Humans; D007194:India; D019041:L-Selectin / Q000235:genetics / Q000378:metabolism; D008297:Male; D009504:Neutrophils / Q000378:metabolism / Q000473:pathology; D017452:Receptors, IgG / Q000096:biosynthesis / Q000235:genetics*; D017086:beta-Thalassemia / Q000235:genetics* / Q000473:pathology
chemical_list: C428200:FCGR3B protein, human; C080568:Fc gamma receptor IIA; D058851:GPI-Linked Proteins; D017452:Receptors, IgG; D019041:L-Selectin