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Volume 129, Issue 4

Cover Figure: CCL2 is involved in pathobiology of systemic mastocytosis. See the article by Canali et al.

WASHINGTON, January 26, 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.

BDR in newly diagnosed patients with WM: final analysis of a phase 2 study after a minimum follow-up of 6 years
Gavriatopoulou et al report excellent long-term outcomes and a favorable toxicity profile with a regimen of bortezomib, dexamethasone, and rituximab as up-front therapy for Waldenström macroglobulinemia.

Recurrent somatic mutations affecting B-cell receptor signaling pathway genes in follicular lymphoma
Krysiak et al detail results of whole-exome sequencing of over 100 patients with follicular lymphoma, describing novel recurrent somatic changes in B-cell receptor signaling pathway and epigenetic modification genes, with implications for targeted therapy.

Rare variants in GP1BB are responsible for autosomal dominant macrothrombocytopenia
From analysis of genomic sequencing of over 1000 patients with rare bleeding and/or platelet disorders, Sivapalaratnam and colleagues report 18 families carrying rare variants of the GP1BB component of the von Willebrand receptor complex that lead to autosomal dominant macrothrombocytopenia.

Angiocrine Bmp2 signaling in murine liver controls normal iron homeostasis
and
Endothelial cells produce bone morphogenetic protein 6 required for iron homeostasis in mice
Two plenary papers in this issue establish that iron homeostasis is regulated by two BMP family proteins produced uniquely by hepatic endothelial cells. Koch and Canali and colleagues used targeted knockout mice to demonstrate that Bmp2 and Bmp6 are critical independent regulators of hepcidin in the control of normal iron homeostasis.

The EMT transcription factor Zeb2 controls adult murine hematopoietic differentiation by regulating cytokine signaling
and
The EMT regulator ZEB2 is a novel dependency of human and murine acute myeloid leukemia
Two complementary studies by J. Li and H. Li and colleagues demonstrate that ZEB2 regulates a critical switch allowing hematopoietic differentiation. ZEB2 suppresses normal granulocyte production, and inhibition in leukemic cells releases the granulocyte differentiation blockade.

How I treat bronchiolitis obliterans syndrome after hematopoietic stem cell transplantation
This large retrospective analysis of over 180 patients with primary ocular adnexal MALT lymphoma presents treatments and follow-up results spanning a period of more than 30 years. The study reaffirms the excellent long-term control of orbital MALT lymphoma with localized radiotherapy.

Diagnosis and management of AML in adults: 2017 ELN recommendations from an international expert panel
Döhner and colleagues summarize updates to the 2010 European LeukemiaNet (ELN) recommendations for the diagnosis and management of acute myeloid leukemia (AML). These updates parallel updates to the World Health Organization recommendations that consider implications of the current understanding of the genetic landscape of AML, new assays for assessing genetics, and minimal residual disease detection.

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.

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