Variability of Iron Load in Patients of Sickle Cell Anaemia (HbSS): A study from Eastern India.

Abstract

Sickle Cell Anaemia (SCA) is one of the commonest haemoglobinopathies due to a point mutation (A→T) of the β-globin gene. Out of five haplotypes, the Arab-Indian haplotype present in India is one of the least severe phenotype and least studied also. It is characterized by lifelong haemolytic anaemia requiring red cell transfusion leading to iron overload. In contrast, there is very high incidence of deficiency of iron, folic acid and vitamin B12. Our objective was to access the Iron status of SCA patients and to find its correlation with various parameters like red cell transfusion, haemolysis and serum hepcidin. This was a cross-sectional study conducted on 208 patients for a period of five years. Complete Blood Count (CBC), iron profile, haemolytic parameters and transfusion requirement were studied and data compared with 52 healthy controls. Few patients (9.6%) revealed significant iron overload (Serum ferritin > 1000 ng/ml). In majority (80.8%) it was either normal or border line raised (300 to 1000 ng/ml) or iron deficiency was noted in a small fraction (9.6%). Frequency of transfusion is the principal factor which positively correlated with level of iron load (p<0.001) while parameters of haemolysis and serum hepcidin level play an insignificant role in this context (p= 0.0634). This study supports the notion that the presentation of SCA patients in India is of "Viscosity - Vaso-Occlusive Crisis (VOC) phenotype" with high incidence of VOC, low haemolytic rate and transfusion requirement. Iron deficiency may be present in SCA patients requiring Iron supplementation. We suggest further studies to establish the role of hepcidin, ferroportin and other factors that control iron absorption in these patients.