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Current Issue
Volume 140,
Issue 14,
October 6 2022

Issue Highlights

Latest in Blood
Free Articles
Plenary Papers
Shruti Chaturvedi; Ana G. Antun; Andrew M. Farland; Ryan Woods; Ara Metjian; Yara A. Park; Gustaaf de Ridder; Briana Gibson; Raj S. Kasthuri; Darla K. Liles; Frank Akwaa; Todd Clover; Lisa Baumann Kreuziger; J. Evan Sadler; Meera Sridharan; Ronald S. Go; Keith R. McCrae; Harsh Vardhan Upreti; Angela Liu; Ming Y. Lim; Radhika Gangaraju; X. Long Zheng; Jay S. Raval; Camila Masias; Spero R. Cataland; Andrew Johnson; Elizabeth Davis; Michael D. Evans; Marshall A. Mazepa; for the United States Thrombotic Microangiopathies Consortium
DOI: 10.1182/blood.2022016640
First Edition
Motoko Koyama; Luke Samson; Kathleen S. Ensbey; Shuichiro Takahashi; Andrew D. Clouston; Paul J. Martin; Geoffrey R Hill
DOI: 10.1182/blood.2022015808
Clinical Trials and Observations
Yifan Zhou; Ruba Shalhoub; Stephanie N. Rogers; Shiqin Yu; Muxin Gu; Margarete A. Fabre; Pedro M. Quiros; Tae-Hoon Shin; Arch Diangson; Wenhan Deng; Shubha Anand; Wenhua Lu; Matthew Cullen; Anna L. Godfrey; Jacobus Preller; Jerome Hadjadj; Emmanuelle Jouanguy; Aurélie Cobat; Laurent Abel; Frederic Rieux-Laucat; Benjamin Terrier; Alain Fischer; Lara Novik; Ingelise J. Gordon; Larisa Strom; Martin R. Gaudinski; Andrea Lisco; Irini Sereti; Thomas J. Gniadek; Andrea Biondi; Paolo Bonfanti; Luisa Imberti; Clifton L. Dalgard; Yu Zhang; Kerry Dobbs; Helen C. Su; Luigi D. Notarangelo; Colin O. Wu; Peter J.M. Openshaw; Malcolm G. Semple; Ziad Mallat; Kenneth Baillie; Cynthia E. Dunbar; George S. Vassiliou
DOI: 10.1182/blood.2022015721


Featured Content

Transplant outcomes in adults with immunodeficiencies and erythroblastic islands as niches for terminal erythropoiesis and granulopoiesis

In this week’s episode we'll review results of the largest retrospective study to date of allogeneic transplantation in adult patients with inborn errors of immunity. We’ll then discuss intriguing new research demonstrating that erythroblastic islands in the bone marrow foster granulopoiesis alongside terminal erythropoiesis—which lays a foundation for better understanding how blood cell production is regulated within these niches.

Hematopoietic stem cell transplantation for adolescents and adults with inborn errors of immunity: an EBMT IEWP study

Albert et al present a retrospective analysis of hematopoietic stem cell transplantation (HSCT) in 320 patients aged 15 to 62.5 years with inborn errors of immunity. Despite a perceived high risk of HSCT in this population, results were excellent, with an overall survival rate of 78% at 1 year. Results were inferior for those with preexisting disease-related complications, suggesting that patients should consider HSCT before developing end-organ morbidities. This study may change the treatment paradigm for these patients.

Erythroblastic islands foster granulopoiesis in parallel to terminal erythropoiesis

Erythrocytes mature within erythroblastic islands (EBIs), where proximity to a central macrophage is thought to facilitate iron transfer and phagocytosis of excluded nuclei. Romano and colleagues transform our understanding of the EBI, using sophisticated methods to enrich for EBI component cells and demonstrate that they also contain myeloid precursors. Further, they demonstrate reciprocal modulation of the balance of myeloid and erythroid maturation, offering a possible explanation for anemia of chronic inflammation.

IL-1β expression in bone marrow dendritic cells is induced by TLR2 agonists and regulates HSC function

Hematopoietic stem and progenitor cells (HSPCs) growth and proliferation are modulated by signals from the bone marrow niche. Li and colleagues characterized the mechanism by which HSPCs respond to inflammatory signaling. They demonstrate that perivascular bone marrow dendritic cells express toll-like receptors (TLRs) and treatment with TLR agonists induces dendritic cell production of IL1β, which in turn increases HSPC expansion.

STAT1 is essential for HSC function and maintains MHCIIhi stem cells that resist myeloablation and neoplastic expansion

Adult hematopoietic stem cells (HSCs) are usually quiescent, although they can be activated in response to acute stresses. Li et al demonstrate that STAT1 has 2 seemingly disparate roles in HSC function. It is necessary for normal repopulation and recovery from myeloablation and in that regard activates quiescent HSCs to proliferate. However, it is also necessary for maintaining a population of HSCs that has high major histocompatibility complex class II (MHCII) expression and is resistant to cycling and apoptosis.

CAR T-cell therapy in highly aggressive B-cell lymphoma: emerging biological and clinical insights

CAR T-cell therapy targeting CD19 has proven highly effective in patients with relapsed and refractory diffuse large B-cell lymphoma (DLBCL), and several products have received approval. Ali et al review the role of this approach for other high-grade non-DLBCL B-cell lymphomas. They discuss how improved understanding of the biological features of these distinct lymphoma subtypes and further testing are needed to define the role of CAR T-cell therapy in their management.

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