Volume 132, Issue 22



November 29, 2018 – 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: EBV-specific T cells maintain remission of EBV lymphoma after allogeneic transplant. See the article by McLauglin et al.

EBV/LMP-specific T cells maintain remissions of T- and B-cell EBV lymphomas after allogeneic bone marrow transplantation
McLauglin et al demonstrate the safety and efficacy of posttransplant administration of Epstein Barr virus–specific T cells targeting latent membrane protein (LMP) in patients with high-risk EBV lymphomas, concluding that treatment improves outcomes, especially in the adjuvant setting.

The current state of sickle cell trait: implications for reproductive and genetic counseling
Pecker and Naik review the clinical, reproductive, and genetic counseling implications of sickle cell trait.

How I treat the blast phase of Philadelphia chromosome–negative myeloproliferative neoplasms
Odenike describes the approach to blastic transformation of Philadelphia chromosome–negative myeloproliferative neoplasms in the context of 3 illustrative cases.

T cell defects in patients with ARPC1B germline mutations account for combined immunodeficiency
ARPC1B is a key factor in actin polymerization, and germline mutations are associated with immunodeficiency. Brigida and colleagues demonstrate that ARPC1B-related immunodeficiency reflects failure of T cells to form the immunologic synapse, confirming the importance of cytoskeletal dynamics for immune function.

Altered patterns of global protein synthesis and translational fidelity in RPS15-mutated chronic lymphocytic leukemia
RPS15 mutations are associated with aggressive and chemorefractory chronic lymphocytic leukemia. Bretones et al demonstrate that mutant RPS15 disrupts protein translation mediated by a range of defects, including reduced ribosome stability, impaired stop codon recognition, and altered proteostasis.

TET2 deficiency leads to stem cell factor–dependent clonal expansion of dysfunctional erythroid progenitors
TET2 is one of the most common mutations in myelodysplastic syndromes and is associated with dyserythropoiesis. Qu et al dissect the pathways by which TET2 deficiency disrupts erythroid maturation.

Novel insights and therapeutic approaches in idiopathic multicentric Castleman disease
Fajgenbaum reviews the current understanding of human herpesvirus–negative multicentric Castleman disease, emphasizing the pathophysiologic role of cytokine pathways that offer potential targets for therapeutic intervention.

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