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Volume 131, Issue 8

 

 

February 22, 2018 – 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.

Cover Figure: Platelet glycoprotein VI contributes to local antibacterial responses to pneumonia in mice.  See the article by Claushuis et al.

Structure-function analysis of ferroportin defines the binding site and an alternative mechanism of action of hepcidin
While it is known that hepcidin increases the ubiquitination and degradation of ferroportin (Fpn), Aschemeyer and colleagues elucidate a novel second mechanism by which hepcidin inhibits Fpn. They demonstrate that hepcidin binding sterically inhibits Fpn’s function as an iron exporter.


A novel role for the macrophage galactose-type lectin receptor in mediating von Willebrand factor clearance
Desialylation of von Willebrand factor (VWF) is known to facilitate its clearance through the Ashwell-Morrell receptor. Ward et al demonstrate that desialylated VWF is also recognized by a macrophage-specific lectin, macrophage galactose-type lectin, contributing significantly to VWF clearance.


Duvelisib, a novel oral dual inhibitor of PI3K-δ,γ, is clinically active in advanced hematologic malignancies
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Activity of the PI3K-δ,γ inhibitor duvelisib in a phase I trial and preclinical models of T-cell lymphoma
Two papers in this issue report promising results from early-stage studies of duvelisib, a novel inhibitor of both PI3K-δ and -γ. In the first, Flinn et al report on dose-finding and expansion studies in over 200 patients with a wide range of relapsed/refractory lymphoma, demonstrating responses in ~50% of patients, including peripheral T-cell lymphoma (PTCL). In the second, Horwitz et al concentrate on the data in PTCL, demonstrating the impact on PI3K signaling and macrophage polarization in PTCL patient–derived xenografts.


Successful outcome following allogeneic hematopoietic stem cell transplantation in adults with primary immunodeficiency
Primary immunodeficiencies (PID) are heterogeneous diseases that are increasingly identified in young adults, and the role of transplant has been controversial because of limited experience and early poor outcomes. Fox and colleagues report excellent results with allogeneic hematopoietic stem cell transplantation in 29 young adult patients with PID, suggesting that transplant should be more readily considered in these patients.


How I treat disseminated intravascular coagulation
Levi and Scully discuss the diagnosis and treatment of disseminated intravascular coagulation through discussion of 4 illustrative cases.

 

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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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