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Volume 133, Issue 15

 

 

April 11, 2019 – 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: Critical and novel insights into the mechanistic etiology of myelofibrosis. See the article by Wakahashi et al.

Vitamin D receptor–mediated skewed differentiation of macrophages initiates myelofibrosis and subsequent osteosclerosis
This Plenary Paper presents novel insights into the pathogenesis of bone marrow fibrosis in JAK2V617F-driven myeloproliferative neoplasia. Drawing on a series of elegant experiments in mouse models, the investigators provide compelling evidence for the critical role of the nuclear vitamin D receptor and bone marrow macrophages in the pathogenesis of idiopathic myelofibrosis.


Characterization and treatment of congenital thrombotic thrombocytopenic purpura
The authors report important clinical information indicating that ~30% of patients with congenital thrombotic thrombocytopenic purpura have persistent symptoms of headache, lethargy, and abdominal pain and are responsive to prophylactic therapy.


Factors associated with durable EFS in adult B-cell ALL patients achieving MRD-negative CR after CD19 CAR T-cell therapy
This is the first study that looks specifically at factors associated with durable event-free survival (EFS), including minimal residual disease (MRD) negativity, in patients with acute lymphoblastic leukemia (ALL) undergoing CD19 chimeric antigen receptor (CAR) T-cell therapy.


Management of acute promyelocytic leukemia: updated recommendations from an expert panel of the European LeukemiaNet
Significant advances have been made recently in the treatment of acute promyelocytic leukemia (APL). This Special Report presents a timely update of European LeukemiaNet’s recommendations for the management of APL..


Genetic drivers of oncogenic pathways in molecular subgroups of peripheral T-cell lymphoma
This study investigated the chromosomal gains and losses of peripheral T-cell lymphoma (PTCL). The results indicate differences in pathogenesis of previously gene expression–defined PTCL subtypes. These molecular pathogenesis-relevant subtypes will likely set a new standard for diagnosis.


Clinical responses and persistent BRAF V600E+ blood cells in children with LCH treated with MAPK pathway inhibition
This paper presents promising results with MAPK-pathway inhibitory treatment in children with neurodegenerative Langerhans cell histiocytosis (LCH).


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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.

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