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Volume 129, Issue 11

Cover Figure: Treatment outcome of monoclonal gammopathy-associated C3 glomerulopathy. See the article by Chauvet et al.

WASHINGTON, March 16, 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.

Treatment of B-cell disorder improves renal outcome of patients with monoclonal gammopathy-associated C3 glomerulopathy
Chauvet et al report the largest study to date on the treatment outcome of patients with monoclonal gammopathy-associated C3 glomerulopathy and demonstrate that renal outcomes are dependent on hematological therapeutic response.

Quantitative stability of hematopoietic stem and progenitor cell clonal output in rhesus macaques receiving transplants
Koelle and colleagues demonstrate that hematopoietic output originates from multiple multipotent clones that are stable over time with little evidence of clonal succession.

Clonal evolution leading to ibrutinib resistance in chronic lymphocytic leukemia
Ahn et al report intriguing clinical and molecular differences between early and late relapsing patients with chronic lymphocytic leukemia following ibrutinib treatment.

miR-125b promotes MLL-AF9–driven murine acute myeloid leukemia involving a VEGFA-mediated non–cell-intrinsic mechanism
Liu and colleagues provide evidence that miR-125b may contribute to leukemogenesis by activating an autocrine loop involving vascular endothelial growth factor-A.

Proteolytic properties of single-chain factor XII: a mechanism for triggering contact activation
The basic mechanisms for contact activation of blood coagulation has remained puzzling. How does factor XII get activated? Using an extensive set of elegant tools, Ivanov et al demonstrate that single-chain factor XII, when exposed to an appropriate surface, can initiate contact activation with the formation of activated factor XII and kallikrein.

Biallelic mutations in DNAJC21 cause Shwachman-Diamond syndrome
Dhanraj et al identify biallelic mutations in DNAJC21 in patients with phenotypical Shwachman-Diamond syndrome (SDS), thus adding an important gene mutation to the list that can cause SDS.

Platelets and von Willebrand factor in atherogenesis
This Blood Spotlight review summarizes current knowledge on the role of platelets and von Willebrand factor in the early stage of atherosclerotic plaque formation, focusing on the putative initiating mechanism driving platelet-endothelial interactions.

Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts
This review guides the reader through the complex field of mastocytosis, which can be a challenging disorder for practicing hematologists. It offers a lucid update with practical information regarding changes in disease classification and treatment concepts.

Sickle cell disease: an international survey of results of HLA-identical sibling hematopoietic stem cell transplantation
In a large data set, Gluckman et al demonstrate the role of HLA-identical sibling marrow transplantation in sickle cell disease with a 5-year overall survival of 95% when performed at an early age (<16 years).

How I treat acquired aplastic anemia
In a How I Treat paper, an experienced investigator presents illustrative clinical vignettes and outlines the current clinical management approach to the patient with aplastic anemia.

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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