SF3B1 mutation but not ring sideroblasts identifies a specific group of myelodysplastic syndrome-refractory cytopenia with multilineage dysplasia

To investigate the clinical implications of SF3B1 and ring sideroblasts in myelodysplastic syndrome-refractory cytopenia with multilineage dysplasia (MDS-RCMD), 238 consecutive MDS-RCMD patients were included in this study. Our results showed that SF3B1 mutation in MDS- RCMD patients identifies a homogenous phenotype characterized by homogeneous hematologic features including a high prevalence of erythroid dysplasia and a high proportion of ring sideroblasts, prolonged survival and reduced leukemic transformation.
Source: Clinical Lymphoma, Myeloma and Leukemia - Category: Hematology Authors: Tags: Original Study Source Type: research

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ConclusionSD should not be considered a poor treatment response and should not be grouped with failure, while HI offers similar prognosis to SD and thus should not be grouped with complete and partial remission. Patients with SD should continue treatment with 5-azacytidine.
Source: Clinical Lymphoma Myeloma and Leukemia - Category: Cancer & Oncology Source Type: research
We examined the relationship between BMF in MDS and survival outcomes, and explored the landscape of somatic gene mutations in the setting of BMF.Patients and MethodsWe retrospectively evaluated 2624 MDS patients for BMF who were divided into 2 groups: grade 0-2 BMF (96%) and severe/grade 3 BMF (4%) based on analysis presented. Commonly MDS tested acquired somatic mutations were also compared between those 2 groups of patients with available next-generation sequencing data.ResultsOnly grade 3 BMF was associated with worse overall survival independent from the Revised International Prognostic Scoring System (IPSS-R) (hazard...
Source: Clinical Lymphoma Myeloma and Leukemia - Category: Cancer & Oncology Source Type: research
Publication date: Available online 16 January 2020Source: Clinical Lymphoma Myeloma and LeukemiaAuthor(s): Bei Xiong, Mei Xue, Yalan Yu, Sanyun Wu, Xuelan ZuoAbstractBackgroundIntegrating the proportion of ring sideroblasts and SF3B1 mutation status is required for diagnosis of sideroblastic subgroups in myelodysplastic syndrome (MDS) as proposed by the World Health Organization 2016 classification. However, the clinical implications of SF3B1 mutation and ring sideroblasts in MDS-refractory cytopenia with multilineage dysplasia (MDS-RCMD) remain unclear.Patients and MethodsClinical and laboratory features in 238 MDS-RCMD p...
Source: Clinical Lymphoma Myeloma and Leukemia - Category: Cancer & Oncology Source Type: research
Purpose of review This review outlines the advancements that have been made in computational analysis for clinical flow cytometry data in hematological malignancies. Recent findings In recent years, computational analysis methods have been applied to clinical flow cytometry data of hematological malignancies with promising results. Most studies combined dimension reduction (principle component analysis) or clustering methods (FlowSOM, generalized mixture models) with machine learning classifiers (support vector machines, random forest). For diagnosis and classification of hematological malignancies, many studies have ...
Source: Current Opinion in Oncology - Category: Cancer & Oncology Tags: CANCER BIOLOGY: Edited by Pierre Hainaut and Sophie Park Source Type: research
Publication date: Available online 31 January 2020Source: Clinical Lymphoma Myeloma and LeukemiaAuthor(s): Roberto Castelli, Luigi Bergamaschini, Thomas Teatini, Luca Cilumbriello, Riccardo Schiavon, Paolo Gallipoli, Giorgio Lambertenghi DeliliesAbstract:IntroductionVon Willebrand factor (vWF) cleaving protease ADAMTS-13 has a key role for maintaining normal size of vWF. A deficiency or dysfunction of vWF cleaving protease is associated with ultra large vWF multimers and thrombotic microangiopathy (TMA). Patients with cancers have reduced levels of vWF cleaving protease. In this pilot study we have evaluated whether or not...
Source: Clinical Lymphoma Myeloma and Leukemia - Category: Cancer & Oncology Source Type: research
Von Willebrand factor (vWF) cleaving protease ADAMTS-13 has a key role for maintaining normal size of vWF. A deficiency or dysfunction of vWF cleaving protease is associated with ultra large vWF multimers and thrombotic microangiopathy (TMA). Patients with cancers have reduced levels of vWF cleaving protease. In this pilot study we have evaluated whether or not deficiencies of ADAMTS-13 were present in myelodysplastic syndromes (MDS). Moreover, we assessed if a reduction in basal levels of ADAMTS-13 may play a role in the prognosis of MDS.
Source: Clinical Lymphoma, Myeloma and Leukemia - Category: Hematology Authors: Tags: Original Study Source Type: research
Publication date: Available online 16 January 2020Source: Clinical Lymphoma Myeloma and LeukemiaAuthor(s): Bei Xiong, Mei Xue, Yalan Yu, Sanyun Wu, Xuelan ZuoAbstractTo investigate the clinical implications of SF3B1 mutation and ring sideroblasts in myelodysplastic syndrome-refractory cytopenia with multilineage dysplasia (MDS-RCMD), 238 consecutive MDS-RCMD patients were included in this study. Our results showed that SF3B1 mutation in MDS- RCMD patients identifies a distinct subgroup characterized by a high prevalence of erythroid dysplasia and a high proportion of ring sideroblasts, prolonged survival and reduced leukem...
Source: Clinical Lymphoma Myeloma and Leukemia - Category: Cancer & Oncology Source Type: research
To investigate the clinical implications of SF3B1 and ring sideroblasts in myelodysplastic syndrome –refractory cytopenia with multilineage dysplasia (MDS-RCMD), 238 consecutive MDS-RCMD patients were studied. SF3B1 mutation in MDS-RCMD patients identified a homogenous phenotype characterized by homogeneous hematologic features, including a high prevalence of erythroid dysplasia and a high propo rtion of ring sideroblasts, prolonged survival, and reduced leukemic transformation.
Source: Clinical Lymphoma, Myeloma and Leukemia - Category: Hematology Authors: Tags: Original Study Source Type: research
Publication date: Available online 14 January 2020Source: Clinical Lymphoma Myeloma and LeukemiaAuthor(s): Megan Melody, Najla Al Ali, Ling Zhang, Hanadi Ramadan, Eric Padron, David Sallman, Kendra Sweet, Jeffrey Lancet, Alan List, John M. Bennett, Rami KomrokjiSummaryBackgroundBone marrow fibrosis (BMF), a poor prognostic factor in myelodysplastic syndromes (MDS), in the context of new risk stratifications of MDS has not been fully explored. Here, we examined the relationship between BMF in MDS and survival outcomes and explored the landscape of somatic gene mutations in the setting of BMF.MethodsWe retrospectively evalua...
Source: Clinical Lymphoma Myeloma and Leukemia - Category: Cancer & Oncology Source Type: research
We examined the relationship between bone marrow fibrosis (BMF) in myelodysplastic syndromes and survival outcomes, and somatic gene mutations in the setting of BMF. Grade 3 BMF was associated with reduced overall survival; however, BMF grade did not affect response to hypomethylating agent or lenalidomide treatment. Complex karyotype, higher bone marrow myeloblasts, lower platelets, and higher rate of elevated lactate dehydrogenase were more often observed in patients with severe BMF. TP53  mutation and SETBP1 were more frequent in patients with grade 3 BMF.
Source: Clinical Lymphoma, Myeloma and Leukemia - Category: Hematology Authors: Tags: Original Study Source Type: research
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