Management of non‐transfusion‐dependent β‐thalassemia (NTDT): The next 5 years
Sign Up to like & getrecommendations! Published in 2020 at "American Journal of Hematology"
DOI: 10.1002/ajh.26055
Abstract: To the Editor: Management of patients with β-thalassemia is undergoing a swift evolution, considering the number of novel agents recently receiving marketing approval or entering clinical development. Although (thankfully) the availability of new treatment options… read more here.
Keywords: ineffective erythropoiesis; thalassemia; management; transfusion ... See more keywords
Age-related inflammatory bone marrow microenvironment induces ineffective erythropoiesis mimicking del(5q) MDS
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DOI: 10.1038/leu.2017.326
Abstract: Anemia is characteristic of myelodysplastic syndromes (MDS). The mechanisms of anemia in MDS are unclear. Using a mouse genetic approach, here we show that dual deficiency of mDia1 and miR-146a, encoded on chromosome 5q and… read more here.
Keywords: mds; erythropoiesis; ineffective erythropoiesis; bone marrow ... See more keywords
Ineffective erythropoiesis in sickle cell disease: new insights and future implications
Sign Up to like & getrecommendations! Published in 2021 at "Current Opinion in Hematology"
DOI: 10.1097/moh.0000000000000642
Abstract: Purpose of review Sickle cell disease (SCD) is a hemolytic anemia caused by a point mutation in the β globin gene leading to the expression of an abnormal hemoglobin (HbS) that polymerizes under hypoxic conditions… read more here.
Keywords: cell disease; erythropoiesis; sickle cell; cell ... See more keywords
SLN124, a GalNac‐siRNA targeting transmembrane serine protease 6, in combination with deferiprone therapy reduces ineffective erythropoiesis and hepatic iron‐overload in a mouse model of β‐thalassaemia
Sign Up to like & getrecommendations! Published in 2021 at "British Journal of Haematology"
DOI: 10.1111/bjh.17428
Abstract: Beta‐thalassaemia is an inherited blood disorder characterised by ineffective erythropoiesis and anaemia. Consequently, hepcidin expression is reduced resulting in increased iron absorption and primary iron overload. Hepcidin is under the negative control of transmembrane serine… read more here.
Keywords: thalassaemia; ineffective erythropoiesis; iron overload; transmembrane serine ... See more keywords
Fetal hemoglobin rescues ineffective erythropoiesis in sickle cell disease
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DOI: 10.3324/haematol.2020.265462
Abstract: While ineffective erythropoiesis has long been recognized as a key contributor to anemia in thalassemia, its role in anemia of sickle cell disease (SCD) has not been critically explored. Using in vitro and in vivo… read more here.
Keywords: cell disease; erythropoiesis; sickle cell; ineffective erythropoiesis ... See more keywords
Ferroportin inhibitor vamifeport ameliorates ineffective erythropoiesis in a mouse model of β-thalassemia with blood transfusions.
Sign Up to like & getrecommendations! Published in 2023 at "Haematologica"
DOI: 10.3324/haematol.2022.282328
Abstract: β-thalassemia is an inherited anemia characterized by ineffective erythropoiesis. Blood transfusions are required for survival in transfusion-dependent β-thalassemia and are also occasionally needed in patients with non-transfusion-dependent β-thalassemia. Patients with transfusion-dependent β-thalassemia often have elevated… read more here.
Keywords: thalassemia; blood transfusions; ineffective erythropoiesis; vamifeport ... See more keywords