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Volume 130, Issue 8

Cover Figure: Novel role of constitutive surface Ig signaling in DLBCL. See the article by Havranek et al.

WASHINGTON, August 24, 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.

Leukofiltration plus pathogen reduction prevents alloimmune platelet refractoriness in a dog transfusion model
Refractoriness to platelets is a notorious problemin the transfusion practice. This work in a caninemodel suggests a ready-to-use strategy based on reduction of white blood cells and a process of pathogen reduction using riboflavin and UV-B irradiation for preventing platelet refractoriness.

Tonic B-cell receptor signaling in diffuse large B-cell lymphoma
This report provides elegant evidence that constitutive tonic B-cell receptor signaling to phosphatidylinositol 3-kinase (PI3K), in the absence of antigen binding, is sufficient and necessary for growth of germinal center B-cell–type diffuse large B-cell lymphoma. It presents novel insights into the role of surface immunoglobulin on these cells and furnishes a rationale for Bruton tyrosine kinase and PI3Kd inhibitor therapy in the disease.

How I treat first relapse of myeloma
The authors review the treatment of relapsed and refractorymultiple myeloma. While various newer drugs are nowavailable in different parts of the world, the authors clarify when and where to use these agents.

NR4A orphan nuclear receptor family members, NR4A2 and NR4A3, regulate neutrophil number and survival
Prince et al provide fundamental insights into the function of the NR4A orphan nuclear receptor protein family in neutrophils. They identify NR4A2 and NR4A3 as critical regulators of neutrophil life span and homeostasis, and demonstrate that they act via a cyclic adenosine monophosphate and protein kinase A–dependent mechanism.

Outcomes of hematopoietic cell transplantation using donors or recipients with inherited chromosomally integrated HHV-6
Hill et al demonstrate the importance of human herpesvirus 6 (HHV-6), one of the ubiquitous viruses in humans, with regard to outcome of allogeneic stem cell transplantation (HCT). For instance, inherited chromosomally integrated HHV-6 impacts profoundly on incidence of acute graft-versus-host disease and cytomegalovirus viremia after HCT.

Expanded repertoire of RASGRP2 variants responsible for platelet dysfunction and severe bleeding
This paper reports causativemutations in the Rap1 activator, calcium and diacylglycerol-regulated guanine exchange factor I (CalDAG-GEFI), in a large cohort of individualswith unexplained severe platelet-type bleeding disorders or their family members. RASGRP2 mutations are identified as prominent causes of familial platelet function disorders. The investigators describe 11 pedigrees with biallelic pathogenic variants in RASGRP2, which encodes CalDAG-GEFI.

High prevalence of myeloid neoplasms in adults with non–Langerhans cell histiocytosis
The authors identify a previously underappreciated high prevalence of myeloid neoplasms in patients with non–Langerhans cell histiocytosis/ Erdheim-Chester disease. These findings may havemajor clinical implications for systematic evaluation for concomitant neoplasms and the use of single-agent RAF-inhibitor therapy in these patients.

 

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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