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Volume 133, Issue 12

 

 

March 21, 2019 – 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, PhD, and Deputy Editor Nancy Berliner, MD.

Cover Figure: Novel form of inherited thrombocytopenia caused by mutations in PTPRJ. See the article by Marconi et al.

Luspatercept improves hemoglobin levels and blood transfusion requirements in a study of patients with β-thalassemia
In a Plenary Paper, Piga and colleagues report improved hemoglobin levels in thalassemia patients treated with luspatercept, a recombinant protein that acts as a ligand trap for transforming growth factor β ligands that suppress late erythropoiesis.


The use of targeted sequencing and flow cytometry to identify patients with a clinically significant monocytosis
Distinguishing between reactive monocytosis and chronic myelomonocytic leukemia (CMML) is challenging. Cargo et al sequenced a panel of genes mutated in myeloid malignancy and demonstrated that patients with defining mutations have a clinical course consistent with CMML regardless of the presence or absence of morphologic dysplasia.


Genome-wide discovery of somatic coding and noncoding mutations in pediatric endemic and sporadic Burkitt lymphoma
Grande et al performed genomic and transcriptomic analysis of Burkitt lymphoma, revealing characteristic patterns associated with Epstein-Barr virus–positive (EBV+) and EBV- subtypes of the disease and identifying pathways that may suggest novel therapeutic targets.


Blood group alters platelet binding kinetics to von Willebrand factor and consequently platelet function
Dunne and colleagues demonstrate that blood type O platelets bind less to von Willebrand factor (VWF) than non–type O platelets, which could amplify the impact of the lower levels of VWF that are seen in individuals with type O blood.


Regulation of globin-heme balance in Diamond-Blackfan anemia by HSP70/GATA1
The phenotype of Diamond-Blackfan anemia (DBA) associated with non-RPS19 mutations is more severe. Rio and colleagues demonstrate that with RPL5 or RPL11 mutations, the DBA phenotype is aggravated by disruption of the balance of globin and heme synthesis.


Mechanisms of immune escape after allogeneic hematopoietic cell transplantation
In a Perspective article that is also this month’s CME article, Zeiser and Vago review the mechanisms of immune evasion that allow leukemia relapse following hematopoietic stem cell transplantation.


How I manage ibrutinib intolerance and complications in patients with chronic lymphocytic leukemia
In the context of 3 clinical cases, Stephens and Byrd discuss the management of the major complications of ibrutinib therapy for chronic lymphocytic leukemia, emphasizing atrial fibrillation, bleeding, infection, and arthralgia.


View this week's complete table of contents

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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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