(WASHINGTON) – 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, and Deputy Editor Nancy Berliner, MD. If you would like a PDF copy of any of the manuscripts highlighted below or would like to request an intervew with the author, please email email@example.com.
Pharmacokinetics and safety of a novel recombinant human von Willebrand factor manufactured with a plasma-free method: a prospective clinical trial, Mannucci et al.
In this week’s issue of Blood, Mannucci and colleagues report on the Phase 1 multicenter clinical trial of plasma-free recombinant human von Willebrand factor (rVWF) in patients with type 3 or severe type 1 von Willebrand disease (VWD). The team of investigators concludes that rVWF is well-tolerated and cleaved normally by the gene ADAMTS13. Because VWF normally stabilizes FVIII and patients with severe VWF deficiency have reduced FVIII, investigators combined rVWF at a fixed ratio with the blood clotting protein human recombinant Factor VIII (rFVIII). In this manuscript, researchers report that rVWF also stabilized endogenous FVIII, suggesting that once FVIII levels are therapeutic, rVWF can be administered by itself.
Acute myeloid leukemia creates an arginase-dependent immunosuppressive microenvironment, Mussai et al.
This week in Blood, Mussai and colleagues report that acute myeloid leukemia (AML) blasts can inhibit the immune system by releasing the enzyme Arginase II. Arginase II is expressed and released from AML blasts and mediates the suppression of T-cell proliferation and the polarization of monocytes into an immunosuppressive M2-like phenotype. In this microenvironment, investigators further document reduced proliferation and differentiation of normal marrow progenitors and report that all of these activities could be modified by inhibiting arginase. These results support the conclusion that AML creates a supportive microenvironment by suppressing immune response and limiting normal progenitor proliferation.
Loss of endothelial protein C receptors links coagulation and inflammation to parasite sequestration in cerebral malaria in African children, Moxon et al.
Cerebral malaria is an important cause of death in African children; however the pathophysiologic basis for the effects of malaria on the brain is not well understood. In this week’s issue of Blood, Moxon and colleagues provide insight into this important question, reporting that in cerebral malaria, infected erythrocytes cause loss of protein C receptors and microvascular coagulopathy. Because these receptors are expressed at low levels in the cerebral vasculature, the investigators postulate that this expression makes the brain unusually sensitive to malaria. Using a novel technique for visualizing microvessels, investigators demonstrate the loss of protein C receptors at sites of adherence of infected erythrocytes that disrupt endothelial protection against fibrin clots in the cerebral microvasculature.
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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