scholarly journals Myelodysplastic Syndrome with Ring Sideroblasts and Multilineage Dysplasia

2020 ◽  
Author(s):  
Keyword(s):  
Myelodysplastic Syndrome ◽  
Multilineage Dysplasia ◽  
Ring Sideroblasts

Clinical Utility of Multiparameter Flow Cytometry in the Diagnosis of 1013 Patients with Suspected Myelodysplastic Syndrome: Correlation to Cytomorphology, Cytogenetic, and Clinical Data.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 595-595
Author(s):  
Wolfgang Kern ◽  
Claudia Haferlach ◽  
Susanne Schnittger ◽  
Torsten Haferlach
Keyword(s):  
Flow Cytometry ◽  
Myelodysplastic Syndrome ◽  
Expression Pattern ◽  
Antigen Expression ◽  
Refractory Anemia ◽  
Multiparameter Flow Cytometry ◽  
Multilineage Dysplasia ◽  
Cytogenetic Aberrations ◽  
Ring Sideroblasts ◽  
Equity Ownership

Abstract Abstract 595 Diagnosis and classification of myelodysplastic syndromes (MDS) is based on cytomorpholgy (CM) and cytogenetics (CG). By the identification of MDS-related aberrant antigen expression multiparameter flow cytometry (MFC) may add important diagnostic information. Examples include an aberrant expression pattern of CD13 and CD16 in granulocytes, an aberrante expression pattern of HLA-DR and CD11b in monocytes and the expression of lymphoid markers on myeloid blasts (Haematologica 2009:94:1124). To evaluate the potential role of MFC in the diagnostic setting of MDS we analyzed 1013 cases with suspected MDS by CM, CG, and MFC in parallel. Cases were classified by CM as refractory anemia (RA, n=31, 3.1%), refractory anemia with ring sideroblasts (RARS, n=27, 2.7%), refractory cytopenia with multilineage dysplasia (RCMD, n=64, 6.3%), refractory cytopenia with multilineage dysplasia and ring sideroblasts (RCMD-RS, n=49, 4.8%), refractory anemia with excess of blasts 1 (RAEB-1, n=133, 13.1%), RAEB-2 (n=81, 8.0%), 5q- syndrome (n=24, 2.4%), chronic myelomonocytic leukemia (CMML, n=65, 6.4%), myelodysplastic syndrome unspecified (MDS-u, n=15, 1.5%), MDS borderline to acute myeloid leukemia (MDS/AML, n=6, 0.6%), MDS/myeloproliferative neplasia overlap (MDS/MPN, n=16, 1.6%), suspected MDS (n=225, 22.2%), reactive condition (n=266, 26.3%), and normal findings (n=11, 1.1%). Cytogenetic findings were normal karyotype (n=768, 75.8%), isolated deletion of long arm of chromosome 5 (del(5q), n=43, 4.2%), isolated aberrations of chromosome 7 (n=14, 1.4%), isolated trisomy 8 (n=30, 3.0%), isolated deletion of long arm of chromosome 20 (del(20q), n=21, 2.1%), complex karyotype (n=23, 2.3%), loss of Y-chromosome (n=43, 4.2%), other aberrations (n=71, 7.0%). Concordance between CM and MFC was 82.0% for diagnostic results in 788 cases with unequivocal CM. 277 of these 788 cases were classified by CM as not having MDS, 13 (4.7%) of which showed MDS-typical features by MFC. Additional 225 cases showed only minor dysplastic features by CM, 51 (22.7%) of which showed clear evidence of MDS by MFC. To further analyze the significance of MDS-related findings by MFC we then focused on cytogenetically aberrant cases without unequivocal MDS by CM. In 6/12 (50.0%) cases with no indication of MDS by CM and MDS-typical cytogenetic aberrations MFC revealed MDS characteristics. In another 11/23 (47.8%) cases with minor dysplastic features by CM and MDS-typical cytogenetic aberrations MFC revealed MDS characteristics. Furthermore, we compared blast counts as determined by CM and MFC and found a strong correlation (p<0.001) although the mean±SD percentage was higher as determined by CM as compared to MFC (4.67±4.18 vs. 3.78±2.97). Frequencies of aberrantly expressed antigens significantly differed between cases rated by CM as MDS (median number of aberrantly expressed antigens: 3), suspected MDS (1), and no MDS (0, p<0.001). In various cases MFC identified MDS-typical aberrant antigen expression in cell compartments not rated dysplastic by CM. Spearman rank correlation confirmed a highly significant relation between the number of aberrantly expressed antigens and IPSS (r=0.409, p<0.001). In 257 cases with data on overall survival (OS) the presence of MDS-related findings (≥3 aberrantly expressed antigens or a blast count >5% in MFC or a reduced side-scatter signal) resulted in significantly inferior 6-year-OS (68% vs. 100% p=0.008). The present analysis clearly demonstrates a diagnostic yield of MFC in addition to cytomorphology and cytogenetics in cases with suspected MDS. Disclosures: Kern: MLL Munich Leukemia Laboratory: Equity Ownership. Haferlach:MLL Munich Leukemia Laboratory: Equity Ownership. Schnittger:MLL Munich Leukemia Laboratory: Equity Ownership. Haferlach:MLL Munich Leukemia Laboratory: Equity Ownership.

scholarly journals Acquired Elliptocytosis as a Manifestation of Myelodysplastic Syndrome with Ring Sideroblasts and Multilineage Dysplasia

2017 ◽  
Vol 2017 ◽  
pp. 1-5 ◽  
Author(s):  
Jacob D. Kjelland ◽  
Denis M. Dwyre ◽  
Brian A. Jonas
Keyword(s):  
Bone Marrow ◽  
Chest Pain ◽  
Myelodysplastic Syndrome ◽  
Bone Marrow Biopsy ◽  
Myelodysplastic Syndromes ◽  
Rare Case ◽  
Elderly Male ◽  
Multilineage Dysplasia ◽  
Ring Sideroblasts

Acquired elliptocytosis is a known but rarely described abnormality in the myelodysplastic syndromes (MDS). Here we report the case of an elderly male who was admitted to the hospital with chest pain, dyspnea, and fatigue and was found to be anemic with an elliptocytosis that had only recently been noted on peripheral smears of his blood. After bone marrow biopsy he was diagnosed with MDS with ring sideroblasts and multilineage dysplasia and acquired elliptocytosis. Here we report a rare case of acquired elliptocytosis cooccurring with MDS with ring sideroblasts and multilineage dysplasia.

scholarly journals SF3B1 mutation identifies a distinct subset of myelodysplastic syndrome with ring sideroblasts

Blood ◽  
2015 ◽  
Vol 126 (2) ◽  
pp. 233-241 ◽  
Author(s):  
Luca Malcovati ◽  
Mohsen Karimi ◽  
Elli Papaemmanuil ◽  
Ilaria Ambaglio ◽  
Martin Jädersten ◽  
...  
Keyword(s):  
Myelodysplastic Syndrome ◽  
Wild Type ◽  
Favorable Prognosis ◽  
Multilineage Dysplasia ◽  
Distinct Subset ◽  
Homogeneous Subgroup ◽  
Key Points ◽  
Ring Sideroblasts

Key Points In MDS with ring sideroblasts, SF3B1 mutation defines a homogeneous subgroup with isolated erythroid dysplasia and favorable prognosis. MDS with ring sideroblasts and wild-type SF3B1 is mainly characterized by multilineage dysplasia and unfavorable prognosis.

Chronic Lymphocytic Leukemia Associated with Myelodysplastic Syndrome with Ring Sideroblasts

2010 ◽  
Vol 103 (8) ◽  
pp. 823-827 ◽  
Author(s):  
Khalil M. Charafeddine ◽  
Georges Y. Ibrahim ◽  
Rami A. Mahfouz ◽  
Ghazi S. Zaatari ◽  
Ziad M. Salem
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
Myelodysplastic Syndrome ◽  
Lymphocytic Leukemia ◽  
Ring Sideroblasts

scholarly journals Heterogeneity of Mesenchymal Stromal Cells in Myelodysplastic Syndrome-with Multilineage Dysplasia (MDS-MLD)

2019 ◽  
Vol 35 (2) ◽  
pp. 223-232 ◽  
Author(s):  
Salar Abbas ◽  
Sanjay Kumar ◽  
Vivi M. Srivastava ◽  
Marie Therese M. ◽  
Sukesh C. Nair ◽  
...  
Keyword(s):  
Myelodysplastic Syndrome ◽  
Mesenchymal Stromal Cells ◽  
Stromal Cells ◽  
Multilineage Dysplasia
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