Introduction: Chronic Myeloid Leukaemia (CML) is a myeloproliferative neoplasm characterised by uncontrolled proliferation of white blood cells and its precursors. At diagnosis, various risk scoring systems have been formulated for risk stratification. In 2016, European Treatment Outcome Study (EUTOS) Long Term Survival (ELTS) score has been formulated most recently. This score was recommended by European LeukaemiaNet in 2020. Objective: The objective of the study was to estimate the frequency of high-risk patients among Chronic Myeloid Leukaemia patients in chronic phase using ELTS scoring system. Materials and Methods: This observational study was conducted among patients at Bangabandhu Sheikh Mujib Medical University (BSMMU) between September 2020 and October 2021. A total of fifty chronic myeloid leukaemia patients were enrolled using purposive sampling technique. Clinical information and haematological parameters were recorded. Bone marrow study was conducted to confirm phase of the disease. ELTS risk score was calculated, and risk stratification was done. Chi-square test was done to find out statistical association between variables. Results: Among study participants, 14% were identified as high-risk cases using ELTS score. 40% of patients were intermediate-risk and 46% were low-risk disease. Presence of hepatomegaly or splenomegaly were significantly more common among high-risk patients. Significantly increased eosinophil and blast percentages and significantly lower haemoglobin level were found in high-risk patients. Conclusion: A fair proportion of the CML patients were identified as high-risk patients. Hepatomegaly, splenomegaly, lower haemoglobin, higher eosinophil and higher blast percentages were identified to be independently associated with higher risk stratification of patients.

Background: Hemoglobin E/β-thalassemia is a common inherited hemoglobin disorder among South Asian countries. The phenotypically diverse presentation of the disease is often attributed to coinheritance of β-globin (HBB) gene mutations. The current study described the phenotype and genetic basis of Hb E/β-thalassemia patients and assessed its relation with clinical severity.Methods: A total of 32 patients were included in this cross-sectional study. Cases were confirmed by using capillary hemoglobin electrophoresis or high-performance liquid chromatography. Those with positive findings were further analyzed with clinical information and ancestral data either from the interview or medical records. Data collection was confined to May 2019 and July 2020. Gene sequencing was performed using Sanger’s sequencing method for mutational analysis, and Mahidol scoring was used to grade clinical severity.Result: A total of 13 heterozygous mutations were identified in the HBB gene. Of all, IVS-1-5 (G>C) (n=17, 53.1%) was the most common, and codon 30 (G>C) (n=4, 12.5%) was the second most common mutations. According to the Mahidol scoring system, 37.5% (n=12) were classified as phenotypically mild, 43.8% (n=14) as moderate and 18.8% (n=6) as severe. The IVS-1-5(G>C) mutation was found to be frequently associated with severe disease and showed no mild form.Conclusion: The present study described the clinical severity and its association with genetic mutations in hemoglobin E/β-thalassemia patients. This finding could guide individually tailored management strategies for this particular group of patients.