(WASHINGTON) – Welcome to “This Week in Blood,” a weekly snapshot of the hottest studies from each week’s issue of Blood, the official journal of the American Society of Hematology (ASH), hand-picked by Blood Editor-in-Chief Bob Löwenberg, MD, and Deputy Editor Nancy Berliner, MD. If you would like a PDF copy of any of the manuscripts highlighted below or would like to request an intervew with the author, please email email@example.com.
Eukaryotic initiation factor 2α phosphorylation mediates fetal hemoglobin induction through a post-transcriptional mechanism, Hahn et al.
Strategies to increase fetal hemoglobin (HbF) levels can ameliorate symptoms and improve the lives of β-hemoglobinopathy patients. While most studies have focused on induction of γ-globin gene expression, the authors of this week’s Plenary Paper investigate whether increasing eukaryotic initiation factor 2α (eIF2α) phosphorylation, a key regulator of protein translation, could enhance HbF posttranscriptionally in human primary erythroid cells. Their results provide convincing evidence that fetal hemoglobin production can be significantly increased through translation, a mechanism which has not previously been targeted. Importantly, the observed increase in HbF accompanied a decrease in HbA. The magnitude of the effect of combined translational and transcriptional approaches can produce the levels of HbF needed to significantly benefit most patients with β-hemoglobinopathies.
Safety and efficacy of imatinib cessation for CML patients with stable undetectable minimal residual disease: results from the TWISTER Study, Ross et al.
This manuscript describes the outcome of ceasing imatinib treatment in 40 patients. The probability of remaining off of imatinib was 47 percent, which compares favorably with previously reported data. In the current study reported in this week’s issue of Blood, investigators report that patients who had received imatinib for at least three years and sustained undetected minimal residual disease (undetectable BCR-ABL transcript levels by RT-qPCR) for a minimum of two years withdrew from therapy with imatinib. The reported treatment-free survival rate was 45 percent with available follow up. Most relapses occurred within the first six months post imatinib withdrawal and none beyond 27 months. This clinically important work confirms the findings previously reported by French investigators in a much smaller patient population.
Common and rare von Willebrand factor (VWF) coding variants, von Willebrand factor levels, and factor VIII levels in African Americans, Johnsen et al.
This work is concerned with the prevalence of von Willebrand Factor (VWF) sequence variants in a large population-based sample of 4,468 African Americans. The investigators used exonic sequence data to assess the association of coding variants with VWF and Factor VIII (FVIII) levels. The data provides further insight into common VWF gene variants that influence the wide range of VWF plasma levels observed in the general population. In this manuscript, the authors identify those VWF variants that have a particular influence on VWF and/or FVIII levels. Sequence variation data are of special interest to those seeking to understand the wide normal range of VWF levels as well as to those undertaking molecular genetic analysis of VWF in patients with von Willebrand disease.
Blood (www.bloodjournal.org), the most cited peer-reviewed publication in the field of hematology, is available weekly in print and online. Blood is the official journal of the American Society of Hematology (ASH) (www.hematology.org), the world’s largest professional society concerned with the causes and treatment of blood disorders.
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