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Volume 130, Issue 7

Cover Figure: CME article:
SF3B1-mutant MDS-RSs originate in multilineage stem cell. See the article by Mortera-Blanco et al.

WASHINGTON, August 17, 2017 – 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.

Coronin 1A, a novel player in integrin biology, controls neutrophil trafficking in innate immunity
This plenary paper by Pick et al reports that the adaptor protein coronin 1A is a novel player in integrin biology. The protein is essential for β2-integrin activation in circulating neutrophils under certain inflammatory conditions. Coronin 1A deficiency hampers adhesion and transendothelial migration of neutrophils and aggravates inflammation.

SF3B1-initiating mutations in MDS-RSs target lymphomyeloid hematopoietic stem cells
Mortera-Blanco et al provide definitive evidence of the origin of myelodysplastic syndrome (MDS) with ring sideroblasts (RS) by tracing mutations in the splicing factor gene SF3B1 back to phenotypic hematopoietic stem cells with multilineage potential. This study shows not only that SF3B1 mutations are early myeloid events in MDS, but also that SF3B1 aberrations are present in pro-B cells.

Daratumumab yields rapid and deep hematologic responses in patients with heavily pretreated AL amyloidosis
The authors present highly encouraging data on the efficacy and safety of daratumumab in relapsed/refractory AL amyloidosis.

Eculizumab treatment and impaired opsonophagocytic killing of meningococci by whole blood from immunized adults
Konar and Granoff describe an elegant series of experiments that address the question of why immunization against meningococcal disease can fail to protect people treated with the complement-blocking antibody eculizumab.

Patient-reported outcomes in hematology: is it time to focus more on them in clinical trials and hematology practice?
In a Perspective, Efficace and colleagues explore the value of patient-reported outcomes in hematologic diseases. They underline the role and practical aspects of patient-reported outcome assessment in clinical research and argue for more use of patient-reported data in clinical trials and routine care.

How I treat patients with aggressive lymphoma at high risk of CNS relapse
In this How I Treat article, the authors describe their personal approach to the controversial issue of central nervous system (CNS) prophylaxis in patients with aggressive lymphoma.

Loss of thymic innate lymphoid cells leads to impaired thymopoiesis in experimental graft-versus-host disease
Dudakov et al present studies of experimental graft-versus-host disease in murine models of allogeneic transplantation, highlighting the importance of dysregulation of the thymus in this context.

Bone marrow failure unresponsive to bone marrow transplant is caused by mutations in thrombopoietin
Seo et al report patients from 3 families with inherited bone marrow failure syndromes due to thrombopoietin (THPO) mutations who are refractory to bone marrow transplantation but respond to romiplostim.

GATA2 haploinsufficiency accelerates EVI1-driven leukemogenesis
This article provides solid evidence that chromosomal rearrangements between 3q21 and 3q26 promote leukemogenesis by inducing not only inappropriate EVI1 expression but also GATA2 haploinsufficiency by recruiting a GATA2-distal hematopoietic enhancer (G2DHE) in the proximity of the EVI1 gene.

 

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.

ASH’s mission is to further the understanding, diagnosis, treatment, and prevention of disorders affecting blood, bone marrow, and the immunologic, hemostatic, and vascular systems by promoting research, clinical care, education, training, and advocacy in hematology.

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