Pre-marriage Sickle Cell Screening Program in South Region of Iran, A Pilot Study on 50 Cases of Sickle Trait

Background: Studies have demonstrated that sickle cell trait can be found in an asymptomatic healthy carrier with normal complete blood count (CBC) and red blood cell (RBC) indices. According to Iranian Ministry of Health bulletin instructions, prenuptial Thalassemia Screening Program (TSP) primarily depends on RBC indices which are measured through a routine CBC. Only when these levels are below the standard values in both couples, hemoglobin electrophoresis is performed. Because of normal values of RBC indices in the sickle cell trait, prenuptial TSP might miss it. Materials and Methods: To approach and prove this hypothesis, we carried out a prospective randomized pilot study of one year duration on 50 known cases of sickle cell trait (the parents of sickle cell and sickle-Thalassemia referral cases to Ahvaz Sickle Cell Center, affiliated to Ahvaz Jondishapour University of Medical Sciences). An informed written consent was obtained from all patients. Complete blood count was done including a blood film, and CBC was done by Helena Automated Cell Counter. Sickle preparation was done by freshly made sodium bisulfate 2%. It was read after one hour for the first time and was then read after 24 hours by macroscopic and microscopic methods. Hemoglobin electrophoresis was done by Helena apparatus. Serum ferritin was measured by Immunoradiometric Assay (IRMA). Iron deficient cases were treated by oral Iron agents. Results: Data of the first screening line showed MCV>80 and MCH>27 in more than 94% and HbA2 value of less than %3 in %100 of sickle cell trait cases. Conclusion: Sickle cell trait with normal indices might be skipped through pre-marriage TSP, and their carrier status is not determined without performing hemoglobin electrophoresis. We recommend mandatory hemoglobin electrophoresis and sickle preparation to be added to the first line of prenuptial Thalassemia Screening Program in the sickle target zones such as the southern parts of Iran.