Hematological Characterization of Beta Thalassemia in Sudanese Patients

Abstract

Thalassemia is common inherited disorder among humans, and they represent a major public health problem in many areas of the world. The study aimed to the measurement of hematological characterization of beta-thalassemia in Sudanese patients. Blood samples from 61 beta-thalassemic patients were collected after written consent form obtained from all participants. The frequency of adults (>18 years) was 45 (73.8%) and children’s (<18 years) was 16 (26.2%); the frequency of male was 27 (44.3%) and 34 were female (55.7%). Hemoglobin estimation and red cell indices were carried out using the automatic blood cell counter Sysmex K × 21N. The results showed that Hb and RBCs indices were varied between mild to moderate and severe decreasing, hemoglobin concentration (Hb) with the mean value of 9.6 g/dL, with minimum value of 6.1 g/dl and maximum of 11.9 g/dl, while RBCs were increased in all patients, mean value 5.2 c/l, mean corpuscular volume mean was 58.9 fl, hematocrit was 30.4, mean corpuscular hemoglobin (MCH) 18.8 pg, mean corpuscular hemoglobin concentration (MCHC) was 31.7pg, and RDW was 18.8%. The method used for hemoglobin electrophoresis was capillary electrophoresis, Hb pattern shows increased HbA2 and HbF, the mean of HbA is 78.3%, HbF is 2.3%, and HbA 2 is 6.5% with the min. value of 3.6% and max. of 12.2%. While the mean of serum iron was 82.75 showed low level, 19 high level, and 35 were normal level. Comparison of hematological analysis (HbA2) in thalassemic patients coexisted with iron deficiency and without result was insignificant difference (p=0.645), this result disagrees with references that say iron deficiency masking HbA2. Nevertheless, the association between HbA2 and HbF revealed a statistically significant difference (p<0.013) and HbA2 with Hb was insignificant (p=0.260). ABSTRACT Thalassemia is a Mendelian autosomal recessive heritable blood disorder, it is a group of genetically determined microcytic, hypochromic anemia’s resulting from a decrease in synthesis of one or more globin chains in the hemoglobin molecule [4]. The most common types are alpha- and beta-thalassemia according to which globin chain is reduced [15]. Beta-thalassemia is classified into three types depending on the severity of symptoms: Thalassemia major also known as Cooley’s anemia [8,16]. Thalassemia intermediate and thalassemia minor, thalassemia major is more severe. The signs and symptoms of thalassemia major appear within the first 2 years of life, children develop life-threatening anemia, and they do not gain weight and grow at the expected rate (failure to thrive) and may develop yellowing of the skin and whites of the eyes (jaundice) [18]. Affected individuals may have an enlarged spleen, liver, heart, and their bones which may be misshapen. adolescents thalassemia major delayed puberty.