Background: Hemoglobinopathies especially thalassaemia and its interaction with HbE and HbS are significant cause of morbidity and mortality in our country. With no feasible treatment, prevention of cases by carrier detection is the only option for successful reduction of the disease burden. VARIANT hemoglobin testing system BIORAD using principle of cation exchange chromatography has been in use and considered as gold standard for carrier detection. The purpose of this study is to compare the efficacy of a different, cheaper instrument; D10 by the same manufacturer BIORAD for carrier detection in beta thalassaemia.Methods: Patients diagnosed as beta thalassaemia carrier by VARIANT hemoglobin testing system (HbA2 value between 4.0-9.0) were retested using D10 instrument and checked for agreement.Results: There was good correlation between VARIANT and D-10 methods with Intraclass correlation coefficient 0.832 (95% Confidence Interval 0.756-0.884). Bland-Altman analysis showed mean bias of +0.3526 (95% CI -0.3958 to +1.101).Conclusions: Although further study is needed with larger sample size for assessment of sensitivity and specificity of D10 instrument, it is evident from this study that this instrument can be an effective and cheaper alternative of VARIANT hemoglobin testing system.
Epithelioid sarcoma, proximal type with osteoclastic giant cells in the pediatric age group has not been reported previously and should be considered in the differential diagnoses of tumors with epithelioid cell morphology and scattered osteoclastic giant cells. Retained INI expression helped to differentiate this tumor from malignant rhabdoid tumor.
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