Volume 130, Issue 19

Cover Figure: New insights into neutrophil integrin activation. See the article by Morikis et al.

WASHINGTON, November 9, 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.

Recombinant ADAMTS-13: first-in-human pharmacokinetics and safety in congenital thrombotic thrombocytopenic purpura
This plenary paper describes promising results of a phase 1 study of recombinant ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) in patients with congenital thrombotic thrombocytopenic purpura that is safe, nonimmunogenic, and well tolerated.

An aberrant NOTCH2-BCR signaling axis in B cells from patients with chronic GVHD
The investigators identify the pathogenic and druggable NOTCH2–B-cell receptor (BCR) axis as a feedback loop in patients with chronic graft-versus-host disease, thus providing a rationale for its therapeutic targeting.

Clonal chromosomal abnormalities appearing in Philadelphia chromosome–negative metaphases during CML treatment
Issa and colleagues report that patients with chronic phase chronic myeloid leukemia (CML) in remission after treatment with tyrosine kinase inhibitors with clonal chromosomal abnormalities (CCA) in Philadelphia chromosome–negative metaphases have a significantly reduced survival as compared to similar patients without CCA.

Selectin catch-bonds mechanotransduce integrin activation and neutrophil arrest on inflamed endothelium under shear flow


CD177 modulates human neutrophil migration through activation-mediated integrin and chemoreceptor regulation
The study on neutrophil integrin activation from Morikis et al describes the molecular dynamics of mechanotransduction which underlie E-selectin–mediated neutrophil rolling and adhesion. The study from Bai et al presents new data on CD177-induced integrin activation in regulating adhesion and transmigration.

The immunophilin FKBP12 inhibits hepcidin expression by binding the BMP type I receptor ALK2 in hepatocytes
Hepatocellular expression of the iron-regulatory hormone hepcidin is influenced by several stimuli, including circulating iron levels, erythropoietic activity, and inflammation. Colucci et al add to our current knowledge of hepcidin regulation and demonstrate a role for the immunophilin FKBP12 in dampening signaling via the BMP type I receptor ALK2.

Nonclassical FCGR2C haplotype is associated with protection from red blood cell alloimmunization in sickle cell disease
Vps34 is a phosphoinositide 3-kinase (PI3K) expressed in megakaryocytes and platelets. Valet et al report the phenotype of a Vps34-null mouse which has microthrombocytopenia and abnormal platelet granules. They demonstrate that, in addition to its influence on platelet production, Vps34 has a previously unexpected role in platelet activation and arterial thrombosis.

Philadelphia chromosome–like acute lymphoblastic leukemia
Philadelphia chromosome (Ph)–like acute lymphocytic leukemia (ALL) has been recognized as an important subtype of pediatric and adult leukemia. The authors provide a comprehensive review of the genomic landscape, clinical features, treatment options and outcome, and genetic risk factors for Ph-like ALL.


This week's complete table of contents

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