Volume 132, Issue 18



November 1, 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: CD44 is a functionally important receptor in advanced mastocytosis. See the article by Mueller et al.

A cloning and expression system to probe T-cell receptor specificity and assess functional avidity to neoantigens
The long-term broad applicability of immunotherapy depends on the identification of tumor-specific neoantigens as functional targets. Hu et al present a novel high-throughput system for the identification and functional characterization of such tumor-specific antigens.

CD44 is a RAS/STAT5-regulated invasion receptor that triggers disease expansion in advanced mastocytosis
Mueller et al demonstrate that CD44 is an important marker of malignant mast cells whose expression correlates with invasiveness of systemic mastocytosis (SM). Depletion of CD44 reduces mast cell tumor growth and invasion in mice, suggesting its potential as a target of therapy for SM.

Fetal γ-globin genes are regulated by the BGLT3 long noncoding RNA locus
Ivaldi and colleagues demonstrate that BGLT3, a long noncoding RNA encoded in the intergenic region downstream of the γ-globin genes, interacts with the locus control region to enhance the stage-specific expression of fetal globin.

How I treat breast implant–associated anaplastic large cell lymphoma
Mehta-Shah and colleagues use two illustrative cases to describe the approach to the treatment of the recently described entity of breast implant–associated anaplastic large cell lymphoma.

Immunodeficiency-associated lymphoproliferative disorders: time for reappraisal?
Natkunam and colleagues propose a new classification of immunodeficiency-associated lymphoproliferative disorders that integrates the viral origin, the underlying immunodeficiency, and the pathologic lesion to better reflect the commonalities of this heterogeneous group of disorders.

Ibrutinib blocks Btk-dependent NF-kB and NFAT responses in human macrophages during Aspergillus Fumigatus phagocytosis
Recent studies suggest that patients treated with ibrutinib are at markedly increased risk of developing pulmonary aspergillosis. Bercusson et al demonstrate that ibrutinib inhibits macrophage responses to Aspergillus fumigatus, blocking neutrophil recruitment and macrophage production of tumor necrosis factor α.

Donor-derived MDS/AML in families with germline GATA2 mutation
Galera et al present 3 families in which young patients with undiagnosed germline GATA2 mutations developed myelodysplastic syndrome (MDS)/acute myeloid leukemia (AML), received family donor transplants, and subsequently developed donor-derived hematologic malignancy, underscoring the need to evaluate young MDS/AML patients for undiagnosed genomic predisposition syndromes.

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