Profile of hemoglobin D trait in West Bengal, India

Hemoglobin (Hb) D Punjab is one of the most commonly observed abnormal hemoglobinopathy worldwide. There was no systematic large published study to investigate the characteristic of Hb D Punjab trait in India. This study was conducted in school and college students, newly married couples and pregnant women after proper counseling in the rural areas of West Bengal state in eastern India. Complete blood count was done by Sysmax Automated Hematology Analyzer KX 21 (Sysmex Corp., Kobe, Japan) and thalassemia testing was done using high-performance liquid chromatography (Variant TM - Bio-Rad Lab., Hercules, CA, USA). A total of 46,139 individuals were screened for hemoglobinopathies. Hb D trait was found in 0.35%. Hypochromia rather than microcytosis is consistent finding in Hb D trait. Anisocytosis is absent in Hb D trait. In almost all (99.37%) cases, Hb D is within 40% of total hemoglobin. This data is likely to be helpful for screening of hemoglobinopathy in resource poor setting.  血红蛋白（Hb) D Punjab是全球最普遍的异常血红蛋白病之一。在印度，之前并没有研究D型特征血红蛋白病的大型出版物。本研究在印度东部的西孟加拉邦的农村地区进行，研究对象是接受过相关咨询后的在校生，大学生，新婚夫妇和孕妇。完整的血细胞计数由日本神户市Sysmex公司的KX21自动化血液分析仪完成，血液测试由美国加州赫拉克勒斯Variant-Bio-Rad实验室通过高效液相色谱法完成。总计46139人被筛查，D型特征血红蛋白病的发现率为0.35%。血红蛋白过少而非小红细胞症，是D型特征血红蛋白病的主要症状。红细胞大小不均没有在D型特征血红蛋白病中被检测出。在所有的情况中（99.37%），D型特征血红蛋白占所有血红蛋白总数的40%。这些数据有利于在资源匮乏地区对血红蛋白病的筛查。</p

The prevalence and characterization of β-thalassemia trait by using high-performance liquid chromatography among the rural population in West Bengal, India

Hemoglobinopathies, common genetic disorders of hemoglobin, can be prevented by population screening and genetic counseling. Identification of these disorders is immensely important epidemiologically and aid in prevention of more serious hemoglobin disorders. Thalassemia is the commonest monogenic disorder in India, which belongs to the thalassemia belt of the world. The present study was undertaken to find out the characteristics of β-thalassemia trait and spectrum of this disorder among the rural population, screened under the hospital-based screening program in West Bengal, a state in eastern part of India. This study was carried out in school and college students, newly married couples and pregnant women after proper counseling in the rural areas of five southern districts of this state. Blood samples were tested by high-performance liquid chromatography. Total 1429 β-thalassemia traits were detected by random screening from this population. The mean value of HbA2 of the study population, having β-thalassemia trait is 4.9%. The prevalence (10.5%) of β-thalassemia trait in West Bengal is higher than other parts of the country. These data are likely to help us in future planning for screening programmes in rural areas of West Bengal, India.  血红蛋白病——常见的血红蛋白遗传疾病，是可以通过人口筛查和遗传咨询的方法来预防的。鉴定此种疾病，对于其传播性及预防其导致的更加严重的血红蛋白疾患有非常重大的意义。印度所处世界地中海贫血带，地中海贫血在印度是最常见的单基因疾病。本研究的目的是通过医院筛查程序来研究印度西孟买邦农村人口有关β-地中海贫血症频谱和特征。该研究在印度南部的五个农村地区进行，研究对象是接受过相关咨询后的在校生，大学生，新婚夫妇和孕妇。血液样本通过高效液相色谱法检测。总计1429例带有β-地中海贫血症特质的样本在这些人口中被随机筛查检测出。在该人口样本中，患有β-地中海贫血症特质的HbA2的平均值为4.9%。在西孟加拉邦，β-地中海贫血症特质的患病率为10.5%，高于印度其他地区。这些数据有利于未来在印度西孟加拉邦农村地区的筛查项目。</p

Once-per-week selinexor, bortezomib, and dexamethasone versus twice-per-week bortezomib and dexamethasone in patients with multiple myeloma (BOSTON): a randomised, open-label, phase 3 trial

This is an accepted manuscript of an article published by Elsevier in The Lancet. The accepted version of the publication may differ from the final published version.Background Selinexor with dexamethasone has demonstrated activity in patients with heavily pretreated multiple myeloma (MM). In a phase 1b/2 study, the combination of oral selinexor with the proteasome inhibitor (PI) bortezomib, and dexamethasone (SVd) induced high response rates with low rates of peripheral neuropathy, the main dose-limiting toxicity of bortezomib. The aim of this trial was to evaluate the clinical benefit of weekly SVd versus standard bortezomib and dexamethasone (Vd) in patients with previously treated MM. Methods This phase 3, randomised, open label trial was conducted at 123 sites in 21 countries. Patients who were previously treated with one to three lines of therapy, including PIs were randomised (1:1) to selinexor (100 mg once-weekly) plus bortezomib (1·3 mg/m2 once-weekly) and dexamethasone (20 mg twice-weekly) [SVd] or bortezomib (1·3 mg/m2 twice-weekly) and dexamethasone (20 mg 4 times per week) [Vd]. Randomisation was done using interactive response technology and stratified by previous PI therapy, lines of treatment, and MM stage. The primary endpoint was progression-free survival (PFS) in the intention-to-treat population. Patients who received at least one dose of study treatment were included in the safety population. This trial is registered at ClinicalTrials.gov, NCT03110562. Findings Between June 2017 and February 2019, 402 patients were randomised: 195 to SVd and 207 to Vd. Median PFS was 13·93 (95% CI 11·73–NE) with SVd versus 9·46 months (8·11–10·78) with Vd; HR 0·70, [95% CI 0·53–0·93]; P=0.0075. Most frequent grade ≥3 adverse events (SVd vs Vd) were thrombocytopenia (77 [40%] vs 35 [17%]), fatigue (26 [13%] vs 2 [1%]), anaemia (31 [16%] vs 20 [10%]), and pneumonia (22 [11%] vs 22 [11%]). Peripheral neuropathy rates (overall, 32·3% vs 47·1%; OR 0·52, [95% CI 0·35-0·79]; P=0.0010 and grade ≥2, 21·0% vs 34·3%; OR 0·50, [95% CI 0·32-0·79]; P=0.0013) were lower with SVd. There were 47 (24%) deaths on SVd and 62 (30%) on Vd. Interpretation Once-weekly SVd is a novel, effective, and convenient treatment option for patients with MM who have received 1-3 prior therapies.Karyopharm Therapeutics Inc