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Volume 129, Issue 15

Cover Figure: Review series: transcription factors in hematopoiesis and hematologic disease. Read this Review Series, introduced by David Bodine.

WASHINGTON, April 13, 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.

Numerous niches for hematopoietic stem cells remain empty during homeostasis
In an important conceptual advance in our understanding of the bone marrow microenvironment that supports hematopoietic stem cells (HSCs), Shimoto and colleagues reveal that the bone marrow contains a large number of empty habitable niches for HSCs.

Abnormal joint and bone wound healing in hemophilia mice is improved by extending factor IX activity after hemarthrosis
This study dealing with the optimal management of joint bleeds in hemophilia B characterizes the natural healing of trauma-induced hemarthrosis in hemophilia B mice, and compares a single injection of recombinant glycoPEGylated factor IX with extended half life with the effects of multiple injections of unmodified recombinant factor IX.

The amyloidogenic light chain is a stressor that sensitizes plasma cells to proteasome inhibitor toxicity
Oliva and colleagues uncover a complex interaction between immunoglobulin AL plasma cells and the amyloidogenic immunoglobulin light chains that they produce.

Improved outcomes for newly diagnosed AL amyloidosis between 2000 and 2014: cracking the glass ceiling of early death

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A phase 2 trial of pomalidomide and dexamethasone rescue treatment in patients with AL amyloidosis
The papers by Muchtar et al and Palladini et al each reflect the significant progress and challenges associated with treatment of systemic light chain (AL) amyloidosis, based on evolving myeloma-derived cytotoxic therapy.

Long-term outcome of hematopoietic stem cell transplantation for IL2RG/JAK3 SCID: a cohort report
Abd Hamid and colleagues describe the long-term outcome of stem cell transplantation in IL2RG/JAK3 SCID severe combined immune deficiency, and report improved B-cell function and improved quality of life in patients who receive pretransplantation conditioning.

Relapse of HHV8-positive multicentric Castleman disease following rituximab-based therapy in HIV-positive patients
Dalla Pria et al investigate whether maintenance therapy has therapeutic value in multicentric Castleman disease, a commonly relapsing systemic illness that is highly associated with HIV infection, and in which the HHV8 virus plays a critical role.

Acquired expression of CblQ367P in mice induces dysplastic myelopoiesis mimicking chronic myelomonocytic leukemia
The E3 ubiquitin ligase CBL gene is commonly mutated in chronic myelomonocytic leukemia (CMML). Nakata et al demonstrate that hematopoietic-specific expression of a mutation in CBL in mice recapitulates the spectrum of key features of human CMML, and furnishes a faithful model system in which to study disease pathogenesis and treatment effects.

Introduction to the review series on transcription factors in hematopoiesis and hematologic disease
Introduced by David Bodine, this set of 6 review articles focuses on 4 hematopoietic transcription factors with critical roles in normal and malignant hematopoiesis. The reviews highlight the fast-moving field of transcription factor biology that is reshaping our understanding of normal and malignant hematology.

The articles in this review series, "Transcription Factors in Hematopoiesis and Hematologic Disease," include the following:

 

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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