TY - JOUR ID - TI - Sickle ß‑globin haplotypes among patients with sickle cell anemia in Basra, Iraq: A cross‑sectional study AU - Mea`ad Kadhum Hassan PY - 2020 VL - 9 IS - 1 SP - 23 EP - 29 JO - Iraq Joural of Hematology المجلة العراقية لامراض الدم SN - 20728069 25432702 AB - BACKGROUND: Sickle cell disease is a monogenic disease with heterogeneous clinical course.Many genetic factors such as inheritance of α‑thalassemia trait and fetal hemoglobin (Hb) level,related to the presence of specific haplotypes, are among the factors that modify disease severity.OBJECTIVES: To identify βS haplotypes of children with sickle cell anemia (SCA) in Basra andassess the association of clinical variables and hematological parameters with different βS haplotypes.PATIENTS AND METHODS: This analytical cross‑sectional study included 62 patients with SCAregistered at Basra Center of Hereditary Blood Disease. In addition to clinical data, blood sampleswere obtained for complete blood count, lactate dehydrogenase and polymerase chain reaction,and Sanger sequencing analysis of HBB gene. Statistical analysis was done using SPSS programversion (23) software.RESULTS: The mean age of studied patients was 7.15 ± 3.81 years, with a male to female ratioof 1:1.7. The most common haplotype was the Arab Indian (AI) in 34 (54.8%) patients, followedby Benin and Senegal haplotypes in 12 (19.4%) patients for each, and an atypical haplotype in4 (6.5%) patients. No significant differences were found in the mean age of diagnosis, the frequencyof vaso‑occlusive crises, blood transfusions and hospitalizations among patients with different βShaplotypes, P > 0.05. However, patients with AI haplotype have significantly higher Hb, red bloodcell count, hematocrit and fetal Hb compared to other haplotypes, P < 0.05.CONCLUSIONS: The AI is the most common haplotype among SCA patients from Basra and it wasassociated with significantly higher Hb, hematocrit, and fetal Hb levels

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