Profile of Iron Overload in Sickle Cell Syndrome
Patients from Eastern India – Correlation with
Common Parameters in Juniper in Open Access Blood Research & Transfusion Journal
Introduction: Eastern India -the melting pot of different heterozygous Sickle cell syndrome. Mostly NTDT, we wanted to study the pattern of iron overload in this heterogeneous yet homogenous group of patients. As there is no such study from this part of the world, it would reveal the true state of iron overload, appropriate test/s and chelation requirement.
Methodology: 152 subjects of Sickle cell syndromes (SS, SB, SE, SD and S Lepore), 5 to 60 years, both sexes, were subjects to crosssectional study, transfusion requirement was calculated retrospectively. Biochemical parameters - hs-CRP and Ferritin and LIC by MRI and correlated to TIL. Chelation requirement was determined.
Observation: All subjects had high hs-CRP (2.67±2,21) and Ferritin (5231.8±1071.8), highest in HbSD Disease and lowest in SB. LIC by MRI R2* was 5.14±3.67 mg/G DLT, highest in S Lepore and SS. Hepatic Fibroscan revealed 13±5kPa and Grade 3-4 fibrosis, mainly in Hb SS patients.
Discussion: Iron overload was noted from second decade. Ferritin gives false high results, when compared to LIC. Even in the absence of significant iron overload, hepatic fibrosis was noted.
Conclusion: Iron overload requiring chelation exists in Sickle Syndrome. Iron overload as estimated by Ferritin is on the higher side, CRP level can be used to exclude inflammatory state. LIC by MRI is the best. Subjects with high CRP and ferritin, have hepatic fibrosis, LIC though normal, regular chelation therapy to control LPI, NTBI and other Oxygen derived free radicals may help.
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