Evaluation of the Relationship Between Hb F Levels and Nucleated Red Blood Cells with Morbidity in Non Transfusion-Dependent Thalassemia Patients

Abstract

Recognition of risk factors of morbidities in patients with β-thalassemia intermedia (β-TI) is an important issue that must be evaluated. Non transfusion-dependent thalassemia patients referred to the outpatient clinic of Shiraz University of Medical Science, Shiraz, South Iran were enrolled in this study between 2013 and 2014. Two peripheral blood smears were prepared for evaluating developmental stage of normoblasts. One hundred and thirty-one patients with ages ranging from 3 to 42 years (mean: 23.35 ± 7.9) were selected. Sixty-seven patients had at least one morbidity (51.1%). Osteoporosis and gallstones were the most common morbidities (33.6 and 24.4%, respectively). In the univariate model, hemoglobin (Hb), ferritin, Hb F, developmental stage of normoblasts and hydroxyurea (HU) therapy did not differ between patients with and without morbidity (p > 0.05) but mean age of patients and mean number of normoblasts were higher in patients with morbidity (p = 0.026 and p = 0.012, respectively). In the regression model, sex and splenectomy status were different between patients with and without morbidity. It seems that females and splenectomy are risk factors for morbidity in non transfusion-dependent thalassemia patients. [Sex: odds ratio (OR) = 2.21, 95% confidence interval (95% CI): 1.04-4.72, p = 0.39. Splenectomy: OR = 3.10, 95% CI: 1.12-8.59, p = 0.029.] This study shows that Hb F level and developmental stage of normoblasts does not effect the incidence of morbidities in non transfusion-dependent thalassemia patients but sex and splenectomy were effective factors in development of morbidities. Thus, splenectomy should be avoided as much as possible in patients with non transfusion-dependent thalassemia.

Association Between Selected Single Nucleotide Polymorphisms in Globin and Related Genes and Response to Hydroxyurea Therapy in Ghanaian Children with Sickle Cell Disease

“…Consistent with the findings from other studies, the observed reduced WBCs, and platelets in the HU+ participants in our study participants confirms the cytoreductive effect of HU. 18 , 19 The findings of higher percentage HbF levels in the HU-treated participants are also consistent with what is generally known about the genetic modifiers of HbF in SCD.…”

“…Interestingly, we observed a slightly reduced Hb level in the HU+ group and speculate that, this could be due to crenation and subsequent lysing of RBC’s since HU plays a role in RBC differentiation. 19 …”