Ring sideroblasts and myelodysplastic syndromes.
نویسندگان
چکیده
منابع مشابه
Myelodysplastic syndromes with ring sideroblasts
Background: The presence of somatic mutations in splicing factor 3b subunit 1 (SF3B1) in patients with Myelodysplastic syndromes with ring sideroblasts (MDS-RS) highlights the importance of the RNA-splicing machinery in MDS. We previously reported the presence of bone marrow (BM) RS in Sf3b1 heterozygous (Sf3b1) mice which are rarely found in mouse models of MDS. Sf3b1 mice were originally engi...
متن کاملNew developments in the understanding and diagnosis of myelodysplastic syndromes with ring sideroblasts☆
mutation, a diagnosis of MDS-RS may be defined with RS in yelodysplastic syndromes (MDS) represent a group of clonal ematopoietic stem cell disorders in which cytopenias of ariable severity are associated with dysplastic changes of ematopoietic precursors, and a higher risk of progression to cute myeloid leukemia (AML).1 Besides dysplasia, the presnce of ring sideroblasts (RS) has long been rec...
متن کاملSplicing factor SF3B1 mutations and ring sideroblasts in myelodysplastic syndromes: a Brazilian cohort screening study
BACKGROUND Myelodysplastic syndromes (MDS) comprise a group of malignant clonal hematologic disorders characterized by ineffective hematopoiesis and propensity for progression to acute myeloid leukemia. Acquired mutations in the gene encoding RNA splicing factor 3B subunit 1 (SF3B1) are highly associated with the MDS subtypes presenting ring sideroblasts, and represent a specific nosological en...
متن کاملSF3B1 haploinsufficiency leads to formation of ring sideroblasts in myelodysplastic syndromes.
Whole exome/genome sequencing has been fundamental in the identification of somatic mutations in the spliceosome machinery in myelodysplastic syndromes (MDSs) and other hematologic disorders. SF3B1, splicing factor 3b subunit 1 is mutated in 60%-80% of refractory anemia with ring sideroblasts (RARS) and RARS associated with thrombocytosis (RARS-T), 2 distinct subtypes of MDS and MDS/myeloprolif...
متن کاملPrevalence and distribution of ringed sideroblasts in primary myelodysplastic syndromes.
In order to determine the prevalence and percentage distribution of ringed sideroblasts in primary myelodysplastic syndromes, the results of Prussian blue staining were analysed in 133 cases. Ringed sideroblasts ranging from 1 to 86% of cells were found in 76 (57%) cases. The cases of primary myelodysplastic syndrome corresponding to the group entitled "acquired idiopathic sideroblastic anaemia...
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ژورنال
عنوان ژورنال: Journal of Clinical Pathology
سال: 1983
ISSN: 0021-9746
DOI: 10.1136/jcp.36.12.1413