(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 interview with the author, please email email@example.com.
Mast cell activation contributes to sickle cell pathobiology and pain, Vincent et al.
The chronic daily pain that adult patients with sickle cell disease often experience, even when not in crisis, is poorly understood. In this week’s issue of Blood, Vincent and colleagues provide insight into potential pathways underlying sickle chronic pain syndromes, demonstrating that mast cell activation and degranulation promote neurogenic inflammation and activation of nociceptors in a mouse model of sickle cell disease. Investigators also report that skin biopsies revealed elevated tryptase levels and increased release of substance P and cytokines. In addition, investigators observed that the use of Imatinib or Cromolyn sodium ameliorated symptoms in the mice, and the latter also increased sensitivity to low doses of morphine. This important study provides insight into the pathophysiology of chronic pain in sickle cell disease, and perhaps more importantly, provides entry points for potential therapeutic intervention with mast cell targeting agents to improve symptoms in patients.
Prognostic value of miR-155 in individuals with monoclonal B-cell lymphocytosis and patients with B-chronic lymphocytic leukemia, Ferrajoli et al.
The role of microRNAs in the pathogenesis of chronic lymphocytic leukemia (CLL) is a focus of major research interest. In this week’s issue of Blood, Ferrajoli and colleagues document several important observations as they present data supporting the clinical importance of miR-155 expression in monoclonal B-cell lymphocytosis (MBL) and CLL. First, investigators note that MBL B cells overexpress miR-155 when compared with normal cells, and the levels of expression are even higher in CLL patients. They also observe that miR-155-containing microvesicles are detectable in both MBL and CLL, and plasma levels of miR-155 are as useful for predicting outcomes as cellular levels. Finally, investigators demonstrate that elevated miR-155 expression levels identified CLL patients with a decreased likelihood of a complete response to chemotherapy. These exciting results suggest for the first time that miR levels can be monitored by a simple plasma assay and can provide important prognostic information about the likelihood of progression from MBL to CLL and the likelihood of a complete response to chemotherapy in CLL.
Factor VIII gene (F8) mutation and risk of inhibitor development in non-severe hemophilia A, Eckhardt et al.
Non-severe hemophilia A is conventionally viewed as a mild disease that does not involve most of the serious, long-term hemophilic complications associated with its more severe forms. However, in a subset of patients, exposure to Factor VIII (F8) can lead to inhibitor development, which profoundly affects the disease phenotype. In this week’s issue of Blood, Eckhardt and colleagues analyzed the association between specific F8 mutations and inhibitor development in more than 1,000 patients with non-severe hemophilia A. Investigators observed that 59 of these patients developed inhibitors, and of the 214 different F8 missense mutations documented in the patients, inhibitor development was associated with a subset of only 19 mutations. This report underscores the importance of F8 genotyping in non-severe hemophilia A for predicting risk of possible F8 therapy.
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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