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Volume 130, Issue 25

Cover Figure: Microtubule structure immunofluorescence staining of cultured fetal liver–derived murine megakaryocytes. See the article by Münzer et al.

December 14, 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.

Five-year results of brentuximab vedotin in patients with relapsed or refractory systemic anaplastic large cell lymphoma
The authors report a 5-year update of brentuximab vedotin treatment of patients with relapsed and refractory systemic anaplastic large cell lymphoma and demonstrate durable remissions. They describe a progression-free survival at 5 years of 57% in 38 complete responders, suggesting the possibility of cure in a subset of these patients.

CK2β regulates thrombopoiesis and Ca2+-triggered platelet activation in arterial thrombosis
The investigators use a mouse model of specific platelet/megakaryocyte deletion of casein kinase 2β (CK2β) to demonstrate that CK2β regulates both platelet formation and activation. (CK2β) deficiency alters megakaryocyte microtubule structure, impairing intracellular Ca2+ release.

c-MPL provides tumor-targeted T-cell receptor-transgenic T cells with costimulation and cytokine signals
The authors describe the preclinical development of c-MPL gene modification of T-cell receptor (TCR)–transgenic T cells to improve the expansion and function of human T cells modified to express a tumor antigen–specific TCR.

Disorders of erythrocyte hydration
This review covers the major disorders of red cell hydration, including the primary disorders (such as the hereditary stomatocytoses) and the most common secondary disorders caused by other gene mutations.

VLITL is a major cross-β-sheet signal for fibrinogen Aα-chain frameshift variants
The authors report a new Aα amyloidogenic fibrinogen pathologic mutation in hereditary amyloidosis, and they explore structural factors that govern amyloid fibril formation and play a role in the clinical consequences of this genetic variant.

Single-cell RNA-seq reveals a distinct transcriptome signature of aneuploid hematopoietic cells
The investigators report single-cell RNA sequencing of bone marrow–derived CD34+ cells from patients with monosomy 7 chromosomal abnormalities. After identifying cells with chromosome 7 aberrations, they found reduced transcription of genes upholding genomic integrity and accumulation of somatic mutations.

Confirmed efficacy of etoposide and dexamethasone in HLH treatment: long term results of the cooperative HLH-2004 study
This paper reports on a pediatric observational treatment study of hemophagocytic lymphohistiocytosis (HLH-2004) and shows that first-line cyclosporine and intrathecal steroids do not add to the therapeutic efficacy of the etoposide-based HLH-94 protocol.

 

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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