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Volume 131, Issue 7

 

 

February 15, 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: Decreased miR-29b leads to oncogene activation through BRD4 in CTCL.  See the article by Kohnken et al.

A landscape of germ line mutations in a cohort of inherited bone marrow failure patients
In a plenary paper that is also this month’s CME article, Bluteau and colleagues report whole-exome sequencing on 179 patients with suspected inherited bone marrow failure and identify causal germ line mutations in nearly half, including a diverse range of known and novel mutations.


Fc-independent immune thrombocytopenia via mechanomolecular signaling in platelets
About 20% of patients with immune thrombocytopenia do not respond to steroids and IV immunoglobulin, a finding more common with antibodies against glycoprotein Ib-IX (GPIb-IX). Quach et al demonstrate that Fc-independent platelet destruction depends on strong antibody binding to the ligand-binding domain of GP1b-IX that can withstand shear stress, allowing receptor unfolding that leads to platelet activation and platelet clearance in the liver.


Prognostic miRNA classifier in early-stage mycosis fungoides: development and validation in a Danish nationwide study
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Diminished microRNA-29b level is associated with BRD4-mediated activation of oncogenes in cutaneous T-cell lymphoma
Two papers in this issue elucidate the role of microRNAs (miRNAs) in the pathogenesis of cutaneous T-cell lymphoma (CTCL) in studies that may allow more individualized approaches to CTCL. In the first, Lindahl and colleagues report on miRNA sequencing in patients with mycosis fungoides (MF) and identify 3 upregulated miRNAs that form a predictive classifier of progression in early-stage MF. In the second, Kohnken et al demonstrate that CTCL is associated with decreased expression of miR-29b, leading to increased BRD4-mediated expression of tumor-associated oncogenes. Furthermore, in a mouse model of CTCL, restoration of miR-29b or suppression of BRD4 prevented CTCL progression.


Altered plasma clot properties increase the risk of recurrent deep vein thrombosis: a cohort study
Cieslik and colleagues characterize in vitro properties of plasma clots that predict recurrence of deep vein thrombosis in vivo in a longitudinal study.


How I use catheter-directed interventional therapy to treat patients with venous thromboembolism
Vedantham and Sista discuss their use of catheter-directed thrombolysis for the treatment of venous thromboembolism in the context of 3 illustrative cases.