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Issue 17, Volume 128

Cover Figure: Molecular basis of the lazy leukocyte syndrome. See the article by Kuhns et al.

WASHINGTON, October 27, 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.

Cytoskeletal abnormalities and neutrophil dysfunction in WDR1 deficiency
Kuhns et al describe the genetic basis for a disease that has mystified investigators for over 45 years, the lazy leukocyte syndrome. The typical symptoms of recurrent infections, stomatitis, a low neutrophil count, and impaired neutrophil motility that characterize this disorder can now be explained by gene mutations in actin-interacting protein 1.

Intron retention resulting from a silent mutation in the VWF gene that structurally influences the 5′ splice site
Yadegari et al report a novel synonymous genetic variant and aberrant von Willebrand factor (VWF) splicing mechanism leading to VWF mRNA intron retention in a patient with type 1 von Willebrand disease.

CD4+ T-cell alloreactivity towards mismatched HLA class II alleles early after double umbilical cord blood transplantation
Lamers and colleagues dissect how CD4+ T cells determine the long-term predominance of one of the cord blood units and the graft-versus-leukemia effect after double umbilical cord transplantation. 

Maternal extracellular vesicles and platelets promote preeclampsia via inflammasome activation in trophoblasts
Preeclampsia is an important and poorly understood disorder. The investigators report a novel connection between platelets, microparticles, and placenta pathology.

inv(16) and NPM1mut AMLs engraft human cytokine knock-in mice
Over the past decade, impressive advances have been made in primary patient-derived xenograft (PDX) modeling of human leukemia. The work reported here demonstrates that humanized cytokine knock-in mice allow high-level, reproducible engraftment of various genotypes of favorable-prognosis acute myeloid leukemia (AML), which have been notoriously resistant to outgrowth in preclinical PDX models.

The CLL-IPI applied in a population-based cohort
This Letter to Blood provides strong external validity for the recently published International Prognostic Index for patients with chronic lymphocytic leukemia (CLL-IPI), an important prognostic tool that will likely be broadly implemented in clinical practice.

 

This week's complete table of contents

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