4.4 Editorial Material

The effects of chronic glucorticoid stimulation on erythropoiesis in Cushing syndrome

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Article Hematology

Patients with hypercortisolemic Cushing disease possess a distinct class of hematopoietic progenitor cells leading to erythrocytosis

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Summary: Although in vitro cell culture experiments suggest that the glucocorticoid receptor (GR) activates stress erythropoiesis, the effects of GR activation on erythropoiesis in vivo remain unclear. This study characterized the phenotype of patients with Cushing disease and found that chronic exposure to excess glucocorticoids leads to erythrocytosis by generating erythroid progenitor cells with a constitutively active GR. Remission of the disease rescues the erythrocytosis but maintains a memory of other prior changes.

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Single-cell profiling of human bone marrow progenitors reveals mechanisms of failing erythropoiesis in Diamond-Blackfan anemia

Deena Iskander et al.

Summary: The study investigates how mutations in ribosomal protein genes affect erythroid progenitor development in Diamond-Blackfan anemia, with RPS-DBA showing erythroid differentiation arrest and RPL-DBA exhibiting relatively preserved abnormal erythroid progenitors and precursors. The findings suggest distinct mechanisms of erythroid failure and phenotype-genotype correlations in DBA, which may aid in identifying therapeutic targets.

SCIENCE TRANSLATIONAL MEDICINE (2021)

Article Medicine, Research & Experimental

Steroid resistance in Diamond Blackfan anemia associates with p57Kip2 dysregulation in erythroid progenitors

Ryan J. Ashley et al.

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Article Endocrinology & Metabolism

Polycythemia as the first manifestation of Cushing's disease

A. Gursoy et al.

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