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Volume 130, Issue 4

Cover Figure: SOX11 promotes homing and invasion of mantle cell lymphoma by regulation of CXCR4 and FAK. See the article by Balsas et al.

WASHINGTON, July 27, 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.

Robust patient-derived xenografts of MDS/MPN overlap syndromes capture the unique characteristics of CMML and JMML
In this week’s plenary paper, Yoshimi and colleagues describe the successful development of a patient-derived xenograft model of chronic myelomonocytic leukemia (CMML) and juvenile myelomonocytic leukemia (JMML) in immunodeficient mice. This model recapitulates the clinical characteristics of the cognate diseases and will provide a much-needed tool for the testing and development of agents to treat these fatal malignancies.

Presenting ADAMTS13 antibody and antigen levels predict prognosis in immune-mediated thrombotic thrombocytopenic purpura
Alwan and colleagues examine factors predicting mortality in the United Kingdom Thrombotic Thrombocytopenic Purpura (TTP) Registry. In 312 episodes of TTP in 292 patients, high antibody and low antigen levels predict increased mortality, as do elevated troponin levels and neurologic dysfunction.

Bendamustine plus rituximab for chronic cold agglutinin disease: results of a Nordic prospective multicenter trial
In a practice-changing study, Berentsen and colleagues report high overall response with significant complete response to a combination of bendamustine plus rituximab in patients with chronic cold agglutinin disease.

Gut microbiota regulate hepatic von Willebrand Factor synthesis and arterial thrombus formation via Toll-like receptor-2
Jäckel et al demonstrate that germ-free and toll-like receptor (TLR) knockout mice both display decreased levels of von Willebrand Factor (VWF) and decreased arterial thrombosis. These studies suggest a pathway that may influence cardiovascular risk via signaling by the gut microbiota through TLRs to increase hepatic synthesis of VWF.

SOX11 promotes tumor protective microenvironment interactions through CXCR4 and FAK regulation in mantle cell lymphoma
Balsas and colleagues explore the role of SOX11 in determining the behavior of mantle cell lymphoma (MCL) cells. They demonstrate that SOX11 regulates MCL cell homing and invasion through regulation of cell adhesion molecules, explaining why SOX11 overexpression in MCL is associated with a more aggressive clinical phenotype.

Thymic epithelial cells require p53 to support their long-term function in thymopoiesis in mice
Rodrigues et al explore the impact of cell-intrinsic p53 expression on murine thymic epithelial cells (TEC), which are critical for T-cell development and induction of tolerance. Analysis of TEC cells with conditional p53 deletion demonstrates that p53 loss impairs the TEC niche, disrupting regulatory T-cell development and increasing autoimmunity, thus defining a new role for p53 in normal T-cell development.

Introduction to a series of reviews on precision hematology
Edited with an introduction by Dr Benjamin L. Ebert, we present a series of reviews focused on precision hematology. This series presents the state of the art usage of genomic techniques for the identification of inherited and acquired mutations guiding the diagnosis and treatment of hematologic disorders, the methods for gathering and storing genomic data, and the ethical considerations in such genomic testing.

The articles in this review series, "Precision Hematology," include the following:

 

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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