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Issue 2, Volume 127

Cover Figure: N-WASP contributes to autoimmunity in Wiskott-Aldrich syndrome. See the article by Volpi et al.

WASHINGTON, January 14, 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.

Intravenous immune globulin and thromboembolic adverse events in patients with hematologic malignancy
Intravenous immunoglobulin (IVIg) is given to patients with chronic lymphocytic leukemia or multiple myeloma to reduce infection secondary to hypogammaglobulinemia. However, there is concern that IVIg may predispose to thromboembolic events (TEE). In this week's Blood , Ammann and colleagues report that a review of the Surveillance, Epidemiology and End Results (SEER) database confirms an increased risk of arterial TEE within 24 hours of IVIg administration. Their findings will prompt reassessment of the risk-benefit ratio of IVIg therapy.

Long-term remissions after FCR chemoimmunotherapy in previously untreated patients with CLL: updated results of the CLL8 trial
Novel targeted agents have transformed the treatment of relapsed chronic lymphocytic leukemia (CLL), but fludarabine, cyclophosphamide, and rituximab (FCR) remain the standard first-line therapy of CLL. In this week's Blood , Fischer and colleagues present long-term follow-up of the CLL8 trial comparing FC vs FCR for untreated CLL. They confirm that FCR remains an excellent therapy for certain subgroups of CLL, with long-term remissions and markedly prolonged overall survival.

N-WASP is required for B-cell-mediated autoimmunity in Wiskott-Aldrich syndrome
Wiskott-Aldrich syndrome (WAS) is caused by mutations in the Was gene, and is characterized by thrombocytopenia, eczema, immunodeficiency, and autoimmunity. In this week's Blood , Volpi and colleagues investigate the mechanism for autoimmunity in the setting of immunodeficiency mediated by loss of Wiskott-Aldrich protein (WASP). They demonstrate that simultaneous conditional deletion of the genes encoding both WASP and its analog neural-WASP (N-WASP) attenuated autoimmune manifestations of WAS, highlighting a potential novel therapeutic target for autoimmune disease.

Genome-wide studies identify a novel interplay between AML1 and AML1/ETO in t(8;21) acute myeloid leukemia
The AML1/ETO fusion protein plays a critical role in the pathogenesis of t(8;21) acute myeloid leukemia, and has been demonstrated to have a dominant-negative effect on transcriptional activation by wild-type AML1. In this week's Blood , Li and colleagues demonstrate by genome-wide chromatin immunoprecipitation-sequencing studies that the interaction between AML1/ETO and wild-type AML1 is much more complicated. They demonstrate overlapping binding of the 2 proteins that regulate whether target genes are up-regulated or down-regulated.

Sex-specific differences in genetic and non genetic determinants of mean platelet volume: results from the Gutenberg Health Study
Mean platelet volume (MPV) is a potential, highly heterogeneous marker of platelet function. In this week's Blood , Panova-Noeva and colleagues perform a comprehensive analysis of genetic and non-genetic factors determining MPV in the Gutenberg Health Study, a cohort of over 15 000 individuals. They demonstrate that MPV is affected by sex-specific genetic determinants as well as other cardiovascular risk factors; high MPV also predicted increased cardiovascular mortality in men. Thus, MPV joins red cell distribution width as an easily detected CBC variable associated with cardiovascular risk.

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.

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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