Aminoacids and Fatty Acids Levels in Patients with Thalassemia Major

Introduction: The red blood cell(RBC) membrane is a complex of lipids an proteins. When thalassemic RBCs are challenged with oxidant stress, incorporation of fatty acids in RBC phospholipids is affected. We investigated the aminoacids, carnitine and fatty acid levels of thalassemic patients.

Material-Method: A total number of 40 thalassemia major patient aged between 7–30 years, who has been taken regular transfusion and deferasirox therapy are included. Serum lipid profil (trigliserit, cholesterol, HDL and LDL) and ferritin levels were measured. Serum levels of aminoacids (valine, leucine/isoluecine, methionine, phenylalanine, tyrosine, aspartat, glutamat, glysine, alanine, ornitine, sitrulline, carnitine and fatty acids (C2 asetil, C3 propionil, C4 butiril, C5:1 tigilil, C5 izovaleril, C4OH3Ohbutiril, C6 hekzanol, C5OH-3 hidroksiizovaleril, C8oktanol, C10 dekanol, C5DC glutaril, C12 dodekenoil, C14.1 metilglutaril, C1 miristoil, C14OH 3 hidoksimiristoil, C16 1-palmitoleil, C16 OH 3 hidroksipalmitoleil, C18 1 oleil, C18 stearoil, C18.1OH 3 hidrosioil) were measured by using tanden-mass spectrometry.

Results: There was found high level of phenylalanine in 7 (17.5%) and fatty acid: c18 oleil 1 patients (2.5%) and low level of glisin in 4 (10%), metionin 1 patients(2.5%), carnitin 1 patients (2.5%) and as fatty acids; C16 palmitol in 11 (27.5%), C3 propiponil in 8 (20%) and C2 asetil 2 patients. (5%). Other amino acids and fatty acids were normal levels. There was significant relationship between fatty acids such as C16 palmitol, C3 propiponil and lipid profil.

Conclusion: Normal lipid profil and fatty acids is changed in thalassemic cells which in turn leads to altered interactions.