Thalassemia is a hereditary blood disorder that results from genetic defects causing deficient synthesis of hemoglobin polypeptide chains. Although thalassemia mostly affects developing countries, there is limited knowledge of its accurate frequency and distribution in these regions. Knowing the prevalence of thalassemia and the frequency of responsible mutations is therefore an important step in the prevention and control program as well as treatment strategies. This study was performed to determine the prevalence and to study the spectrum of gene deletions that are responsible in α-thalassemia in Kelantan, located in northeastern Malaysia. A total 400 first-time blood donors from multiple areas of donation centre were chosen randomly. The presence of three types of α-thalassemia gene deletion in southeast Asian population which were -SEAdeletion, -α
3.7 rightward deletion, and -α
4.2 leftward deletion was detected by using multiplex PCR method. 37 (9.25%) of blood donors were confirmed to have α-thalassemia deletion types. 34 (8%) were heterozygous for α3.7 deletion, 1 (0.25%) was heterozygous for α4.2 deletion, and 2 (0.5%) were heterozygous for SEA type deletion. Alpha-thalassemia-2 with 3.7 deletion was the most common determinant detected in Kelantan Malay compared to other ethnic groups. It has been noted that alpha-thalassemia-2 with 3.7 deletion is the most common type of α-thalassemia throughout the world.
Immunoglobulin gene rearrangement, which occurs in almost all haematological malignancies of B-cell lineage, also presents in a very small proportion of T-cell or myeloid malignancies.
The rate of near misses in transfusion is important as it indicates situations with the potential of adverse outcome. The aim of this study was to assess the frequency of mislabeled and miscollected samples received by our transfusion medicine unit. This study was conducted from January to December 2009 in Transfusion Medicine Unit, Hospital Universiti Sains Malaysia. The total number of near-miss events reported and analysed over the 1-year period was 178 (0.40%). All mislabeled and miscollected samples and its location cases were identified. Mislabeled and miscollected (WBIT) samples were 66.3% and 33.7%, respectively. The highest number of mislabeled and miscollected samples was from accident and emergency unit and medical ward, respectively. Continuous monitoring and analysis of near misses data should be mandatory in order to improve the safety of transfusion.
The association of the severe form of thalassemia with complications of blood transfusion, iron overload, bony deformity and gall stone is well described. Currently, due to improvement in the way of managing thalassemia, most of thalassemia patients survive longer. However more new complications had been observed.Increased incidence of thromboembolism among thalassemia patients had triggered various studies done to determine hypercoagulable state among thalassemia patients. Several factors for the hypercoagulable state had been identified such as RBC membrane disruption, chronic platelet activation and defect in coagulation pathway. This study was carried out to determine the level of Protein C, protein S, free protein S and antithrombin among thalassemia patients and to compare the level of Protein C, protein S, free Protein S and antithrombin level between thalassemia patients and healthy control. This was a case control study done at Hospital Universiti Sains Malaysia. Thirty six thalassemia patients who came for regular blood transfusion and 20 healthy blood donors for normal control were recruited. Blood samples were collected and analyzed for Protein C, protein S, free protein S and antithrombin using ACL Elite Pro. The result showed mean Protein C and free protein S levels were significantly lower (54.8 ± 13.2% and 70.0 ± 12.1% respectively) in thalassemia patients compared to age-matched normal control (94.1 ± 16.3% and 99.8 ± 17.3% respectively), whereas mean total protein S (54.8 ± 13.2% and 94.1 ± 16.3%; respectively) and antithrombin (70.0 ± 12.1% and 99.8 ± 17.3%; respectively) level were similar. In conclusion there was a significantly decreased Protein C and free protein S in thalassemia patients which might suggests hypercoagulable state in thalassemia patients. Since there are a lot of similarities in finding from other studies, we believe that many more study to look for other parameters contributing to hypercoagulable state in thalassemia patients is needed.
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