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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 Fc  γ  R polymorphisms (Fc  γ  RIIA and Fc  γ  RIIIB) are considered to influence clinical presentation. This study focuses on distribution of Fc  γ  R 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. Fc  γ  Rs 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 Fc  γ  RIIA and the NA1/NA1 genotype of Fc  γ  RIIIB 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).   Conclusion . The two Fc  γ  R polymorphisms had significant association with variable phenotypes of sickle cell disease. The expression of CD62L decreased in our patients indicating activation of neutrophils.

The Fc Receptor Polymorphisms and Expression of Neutrophil Activation Markers in Patients with Sickle Cell Disease from Western India