Volume 133, Issue 16



April 18, 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: Disrupted filamin A/αIIbβ3 interaction induces macrothrombocytopenia. See the article by Donada et al.

Disrupted filamin A/αIIbβ3 interaction induces macrothrombocytopenia by increasing RhoA activity
Donada et al used induced pluripotent stem cells to dissect the mechanism by which mutations in the X-linked FLNA gene, which encodes filamin A, cause RhoA activation and macrothrombocytopenia.

Phase 2 study using oral thalidomide-cyclophosphamide-prednisone for idiopathic multicentric Castleman disease
Zhang and colleagues report impressive phase 2 results for an oral regimen of thalidomide, cyclophosphamide, and prednisone for the treatment of siltuximab-resistant idiopathic multicentric Castleman disease.

PHF6 regulates hematopoietic stem and progenitor cells and its loss synergizes with expression of TLX3 to cause leukemia
Somatic mutations in PHF6 occur frequently in hematopoietic malignancy, often in conjunction with increased TLX3. McRae et al used elegant mouse experiments to demonstrate that PHF6 is a tumor suppressor gene, loss of which synergizes with TLX3 overexpression to cause leukemia.

The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas
Willemze and colleagues update the World Health Organization–European Organisation for Research and Treatment of Cancer (WHO-EORTC) classification of primary cutaneous lymphomas in this month’s CME article. The authors emphasize newly recognized entities and newly defined molecular features.

Taking a "BiTE out of ALL": blinatumomab approval for MRD-positive ALL
Blinatumomab, a bispecfic T-cell engager that was recently approved by the US Food and Drug Administration for treatment of minimal residual disease (MRD)–positive acute lymphoblastic leukemia (ALL), is the first approved therapeutic indication based on MRD assessment. Curran and Stock discuss the role of this agent in the treatment of ALL, emphasizing pressing questions that remain to be resolved.

View this week's complete table of contents

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