Hippo kinase loss contributes to del(20q) hematologic malignancies through chronic innate immune activation
Deletions of chromosome 20q are frequent abnormalities in myelodysplastic syndrome (MDS) and myeloproliferative neoplasms. Stoner et al identify STK4, which encodes Hippo kinase MST1, as the candidate gene from the deleted region that, when deleted, promotes features of MDS and induces myelofibrosis in the presence of JAK2V617F through modulation of inflammatory pathways.
Low factor V level ameliorates bleeding diathesis in patients with combined deficiency of factor V and factor VIII
Combined factor V and factor VIII deficiency is a rare disorder associated with relatively mild bleeding diathesis. Shao and colleagues elucidate the double role of factor V as both a pro- and anticoagulant protein, demonstrating that decreased factor V may ameliorate factor VIII deficiency through decreasing the level of tissue factor pathway inhibitor.
Mismatched related vs matched unrelated donors in TCRαβ/CD19-depleted HSCT for primary immunodeficiencies
Laberko et al report excellent survival outcomes for patients receiving TCRαβ/CD19-depleted hematopoietic stem cell transplantation (HSCT) for primary immunodeficiencies, demonstrating comparable results with mismatched related and matched unrelated donors.
Venetoclax and obinutuzumab for frontline treatment of chronic lymphocytic leukemia
In a Blood Spotlight, Stephens reviews the evidence supporting the administration of venetoclax and obinotuzumab for frontline treatment of chronic lymphocytic leukemia and places the combination in the context of other accepted up-front therapies.
Heme-regulated eIF2αkinase in erythropoiesis and hemoglobinopathies
Chen and Zhang review the role of eukaryotic initiation factor 2α (eIF2α) in regulating the balance between protein synthesis and iron availability as part of the integrated stress response in erythroid cells.
Eltrombopag for immune thrombocytopenia secondary to chronic lymphoproliferative disorders: a phase 2 multicenter
Visco and colleagues report excellent response to eltrombopag in immune thrombocytopenia in the setting of chronic lymphoproliferative disease.
