Volume 133, Issue 4



January 24, 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: Impaired dendritic cell–CD4 T cell interaction leads to myeloproliferation. See the article by Humblet-Baron et al.

Structure and dynamics of the platelet integrin-binding C4 domain of von Willebrand factor


The von Willebrand factor Tyr2561 allele is a gain-of-function variant and a risk factor for early myocardial infarction
Two related papers provide novel insights into von Willebrand factor (VWF) platelet integren-binding dynamics. Xu et al solve the crystal structure of the VWF C4 domain, which binds to platelet integrins. Schneppenheim et al demonstrate that a gain-of-function variant of VWF is a risk factor for early myocardial infarction. The mutation alters the complex subdomain structure of the C4 region rather than altering platelet binding.

How I treat cancer-associated venous thromboembolism
Through the discussion of 3 illustrative cases, Kraaijpoel and Carrier describe the approach to the management of cancer-associated venous thrombosis, including the approach to acute deep vein thrombosis, incidental pulmonary embolism, and catheter-associated thrombosis.

Murine myeloproliferative disorder as a consequence of impaired collaboration between dendritic cells and CD4 T cells
Depletion of dendritic cells (DCs) is associated with myeloproliferation in both mice and humans and has been thought to be a compensatory response to the numerical DC deficiency. Humblet-Baron and colleagues overturn that paradigm, demonstrating that disrupting the functional activation of CD4 T cells by DCs, regardless of DC cell number, leads to myeloproliferative disease.

Ibrutinib for the treatment of Bing-Neel syndrome: a multicenter study
Castillo et al report excellent responses to ibrutinib in 28 patients with Bing-Neel syndrome, a phenomenon associated with central nervous system involvement with Waldenström macroglobulinemia.

Regulation of SOX11 expression through CCND1 and STAT3 in mantle cell lymphoma
Increased SOX11 expression is a hallmark of typical mantle cell lymphoma (MCL). Mohanty and colleagues demonstrate divergent roles for CCND1 and STAT3 in regulating SOX11 expression in MCL, providing insights into pathogenesis and potential therapeutic implications.

The Gp1ba-Cre transgenic mouse: a new model to delineate platelet and leukocyte functions
Nagy et al describe a new mouse model for analyzing platelet function through gene knockout studies. Unlike the platelet factor 4-Cre recombinase model, which has been widely used, the current model has more highly restricted megakaryocyte/platelet-specific expression.

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