TO MONITOR TREATMENT OF A LARGE COHORT OF PATIENTS WITH β-THALASSAEMIA MAJOR, INTERMEDIA & SICKLE CELL ANAEMIA IN ORDER TO ESTABLISH THE RESPONSE TO TRANS-RESVERATROL AND THE ASSOCIATED ELEMENTS.

Though several genetic, non-genetic and pharmacological factors reported to influence the Trans-Resveratrol response in different early studies, the response to Trans-Resveratrol is significantly different among good, moderate and non-responders irrespective of the IVS I-5 (GC), the common beta mutation here and even among other β0 or β+thalassaemia mutations. Here we study whether HbF level has any relation to beta variants responding to Trans-Resveratrol therapy, it has been shown that even among good responders in some cases (8.39 %) patients are not showing high HbF values ( < 20% HbF values are taken). In our study (among 220 patients), Trans-Resveratrol therapy completely replace blood transfusion in as expected in this Eastern part of India, the predominant β-thalassemia defect is the IVSI-5(GC), the most frequent β0-thal mutation in the area. This mutation is found in 87.66% of the CR patients and in 86.66% of the PR / NR patients. The next represented defects are the β+-thalassemia mutations like Cod 8/9, Fr. 41/42, Cod 15, Cod 30 etc. The common IVSI-5 (GC) mutation in beta thalassemia patients (either in homozygous or heterozygous form) is present in 31.5% among the good responders (CR) while it is present in 66.66% among PR / NR (the moderate responders / non-responders) and in HbE-beta patients present in 73.28% among good responders (CR) while in 33.33% among moderate / non responders (PR / NR).