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Issue 11, Volume 127

Cover Figure: Lysyl oxidase-associated and risk of thrombosis.See the article by Matsuura et al.

WASHINGTON, March 17, 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.

Prevalence, clinical characteristics, and prognosis of GATA2-related myelodysplastic syndromes in children and adolescents
Germline mutations in GATA2 have been described in several congenital disorders including MonoMAC and Emberger syndromes. In their plenary paper in this issue of Blood, Wlodarski and colleagues demonstrate that children and adolescents diagnosed with myelodysplastic syndrome (MDS) and monosomy 7 commonly harbor germline mutations in GATA2. The overall frequency of germline GATA2 mutations in children with primary MDS is 7%, and it is 15% in those presenting with advanced disease. GATA2 is implicated as a common predisposing factor for the development of primary advanced MDS both in childhood and in adolescence.

Immunovirotherapy with vesicular stomatitis virus and PD-L1 blockade enhances therapeutic outcome in murine acute myeloid leukemia
Oncolytic virotherapy is an emerging therapeutic modality that employs live, replicating viruses to target, infect, and kill cancer cells in vivo. In this issue of Blood, Shen et al demonstrate that the VSV-IFNβ-NIS oncolytic virus has powerful antileukemia activity; this activity is enhanced when combined with an anti–PD-L1 antibody.

Pomalidomide reverses γ-globin silencing through the transcriptional reprogramming of adult hematopoietic progenitors
One of the major goals of hemoglobinopathy research has been to devise improved pharmacologic strategies for the induction of fetal hemoglobin (HbF) in individuals with sickle cell disease and β-thalassemia. In this issue of Blood, Dulmovits and colleagues report that pomalidomide, a drug approved for treatment of multiple myeloma, induces HbF production by decreasing levels of several key transcriptional repressors of fetal globin gene expression.

Recurrent venous thromboembolism and abnormal uterine bleeding with anticoagulant and hormone therapy use
Martinelli et al provide reassuring data that women taking oral anticoagulant therapy for venous thromboembolism may use estrogen or progestin hormonal therapy to control the menstrual bleeding without increased risk for recurrent thromboembolism.

A lymphomagenic role for HIV beyond immune suppression?
Dolcetti et al provide a comprehensive and timely review of the direct connection between HIV and lymphomagenesis, a topic that has been largely neglected.

Strategies that delay or prevent the timely availability of affordable generic drugs in the United States
Jones and colleagues, in an enlightening Blood Forum article, describe the variety of strategies used to delay the availability of generic drugs. This topic will be of ever-increasing relevance, given the number of drugs coming off patent and the consequent development of generics.

Arf6 controls platelet spreading and clot retraction via integrin αIIbβ3 trafficking
In a departure from studies of classic platelet function, Huang and colleagues turn their attention to endocytosis and show that ADP-ribosylation factor 6 (Arf6) plays a key role in fibrinogen engulfment. The authors demonstrate that Arf6-dependent endocytosis is restricted to fibrinogen. Arf6 modulates trafficking of αIIbβ3 integrins in platelets with consequences for platelet spreading and clot retraction.

The hematopoietic oncoprotein FOXP1 promotes tumor cell survival in diffuse large B-cell lymphoma by repressing S1PR2 signaling
Inactivating mutations in the S1PR2 promoter have been associated with the germinal center diffuse large B-cell lymphoma subtype. Flori et al identify S1PR2 as a tumor suppressor that is transcriptionally silenced by FOXP1 in the aggressive, activated B cell subtype.

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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