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Issue 25, Volume 127

Cover Figure: Relationship between CALR mutations and acquired MPO deficiency in MPN. See the article by Theocharides et al.

WASHINGTON, June 23, 2016 – 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.

Heterozygous STAT1 gain-of-function mutations underlie an unexpectedly broad clinical phenotype
Toubiana and colleagues offer the fullest clinical picture to date of the complex primary immunodeficiency caused by heterozygous gain-of-function mutations of signal transducer and activator of transcription 1 (STAT1).

Matriptase activation connects tissue factor–dependent coagulation initiation to epithelial proteolysis and signaling
An expanding body of literature points to an important role of coagulation protein-mediated cell signaling by the activation of protease-activated receptors (PARs). Factor VIIa (FVIIa) and FXa are only weak activators of PAR2. Le Gall et al show that PAR2 activation in coagulation initiation is mediated by the protease matriptase in epithelial cells.

How I treat mycosis fungoides and Sézary syndrome
The treatment of cutaneous T-cell lymphomas is challenging. In their How I Treat article, Whittaker and colleagues bring unique areas of multidisciplinary expertise and offer clinical practice guidelines for the treatment of mycosis fungoides and Sézary syndrome.

Homozygous calreticulin mutations in patients with myelofibrosis lead to acquired myeloperoxidase deficiency
Four decades after the first description of acquired myeloperoxidase deficiency in myeloproliferative neoplasia, this paper presents convincing evidence of its induction by calreticulin gene mutations.

Dynamic monitoring of circulating tumor DNA in non-Hodgkin lymphoma
This state-of-the-art Blood Spotlight review highlights the emerging interest in the use of circulating tumor DNA assessment of treatment response and outcomes in patients with B-cell lymphoma, as well as individual patient tumor heterogeneity and clonal evolution.

The BCL2 selective inhibitor venetoclax induces rapid onset apoptosis of CLL cells in patients via a TP53-independent mechanism

BCR signaling inhibitors differ in their ability to overcome Mcl-1–mediated resistance of CLL B cells to ABT-199
The BCL2 inhibitor venetoclax is one of the promising novel therapeutic agents in chronic lymphocytic leukemia (CLL). Anderson et al demonstrate that venetoclax induces apoptosis in CLL cells independently of TP53 function in vitro and in vivo, and they indicate a role for BH3 profiling in determining a patient’s response to treatment. In a second paper, Bojarczuk et al show that BCR signaling impacts venetoclax sensitivity by Mcl-1 overexpression and suggest a clinically applicable approach to target this phenomenon through inhibiting SYK.

 

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.

blood® is a registered trademark of the American Society of Hematology.