Matches in SemOpenAlex for { <https://semopenalex.org/work/W3120602004> ?p ?o ?g. }
- W3120602004 endingPage "394" @default.
- W3120602004 startingPage "379" @default.
- W3120602004 abstract "Disease Overview Ring sideroblasts (RS) are erythroid precursors with abnormal perinuclear mitochondrial iron accumulation. Two myeloid neoplasms defined by the presence of RS, include myelodysplastic syndromes with RS (MDS-RS) and MDS/myeloproliferative neoplasm with RS and thrombocytosis (MDS/MPN-RS-T). Diagnosis MDS-RS is a lower risk MDS, with single or multilineage dysplasia (MDS-RS-SLD/MLD), <5% bone marrow (BM) blasts, <1% peripheral blood blasts and ≥15% BM RS (≥5% in the presence of SF3B1 mutations). MDS/MPN-RS-T, now a formal entity in the MDS/MPN overlap syndromes, has diagnostic features of MDS-RS-SLD, along with a platelet count ≥450 × 109/L and large atypical megakaryocytes. Mutations and Karyotype Mutations in SF3B1 are seen in ≥80% of patients with MDS-RS-SLD and MDS/MPN-RS-T, and strongly correlate with the presence of BM RS; MDS/MPN-RS-T patients also demonstrate JAK2V617F (50%), DNMT3A, TET2 and ASXL1 mutations. Cytogenetic abnormalities are uncommon in both. Risk Stratification Most patients with MDS-RS-SLD are stratified into lower risk groups by the revised-IPSS. Disease outcome in MDS/MPN-RS-T is better than that of MDS-RS-SLD, but worse than that of essential thrombocythemia (MPN). Both diseases are associated with a low risk of leukemic transformation. Treatment Anemia and iron overload are complications seen in both and are managed similar to lower risk MDS and MPN. Luspatercept, a first-in-class erythroid maturation agent is now approved for the management of anemia in patients with MDS-RS and MDS/MPN-RS-T. Aspirin therapy is reasonable in MDS/MPN-RS-T, especially in the presence of JAK2V617F, but the value of platelet-lowering drugs remains to be defined." @default.
- W3120602004 created "2021-01-18" @default.
- W3120602004 creator A5016216479 @default.
- W3120602004 creator A5072463255 @default.
- W3120602004 date "2021-01-28" @default.
- W3120602004 modified "2023-10-18" @default.
- W3120602004 title "Myelodysplastic syndromes with ring sideroblasts ( <scp>MDS‐RS</scp> ) and <scp>MDS</scp> /myeloproliferative neoplasm with <scp>RS</scp> and thrombocytosis ( <scp>MDS/MPN‐RS‐T</scp> ) – “ <scp>2021</scp> update on diagnosis, risk‐stratification, and management”" @default.
- W3120602004 cites W1757579637 @default.
- W3120602004 cites W1819336302 @default.
- W3120602004 cites W187238933 @default.
- W3120602004 cites W1959044271 @default.
- W3120602004 cites W1963865821 @default.
- W3120602004 cites W1966948071 @default.
- W3120602004 cites W1967997373 @default.
- W3120602004 cites W1969121682 @default.
- W3120602004 cites W1970566157 @default.
- W3120602004 cites W1972429414 @default.
- W3120602004 cites W1972437605 @default.
- W3120602004 cites W1974371672 @default.
- W3120602004 cites W1977142594 @default.
- W3120602004 cites W1978522934 @default.
- W3120602004 cites W1978538135 @default.
- W3120602004 cites W1979233636 @default.
- W3120602004 cites W1979820704 @default.
- W3120602004 cites W1983215575 @default.
- W3120602004 cites W1986544757 @default.
- W3120602004 cites W1991484260 @default.
- W3120602004 cites W2005150790 @default.
- W3120602004 cites W2006383772 @default.
- W3120602004 cites W2007487257 @default.
- W3120602004 cites W2014903824 @default.
- W3120602004 cites W2017299352 @default.
- W3120602004 cites W2020197807 @default.
- W3120602004 cites W2021333892 @default.
- W3120602004 cites W2021572674 @default.
- W3120602004 cites W2022635162 @default.
- W3120602004 cites W2029595590 @default.
- W3120602004 cites W2031212222 @default.
- W3120602004 cites W2033556677 @default.
- W3120602004 cites W2038795176 @default.
- W3120602004 cites W2041981155 @default.
- W3120602004 cites W2064205563 @default.
- W3120602004 cites W2064409835 @default.
- W3120602004 cites W2065358152 @default.
- W3120602004 cites W2070152906 @default.
- W3120602004 cites W2073889874 @default.
- W3120602004 cites W2075352689 @default.
- W3120602004 cites W2077345359 @default.
- W3120602004 cites W2083481160 @default.
- W3120602004 cites W2085128863 @default.
- W3120602004 cites W2086974150 @default.
- W3120602004 cites W2094456737 @default.
- W3120602004 cites W2095263174 @default.
- W3120602004 cites W2097692898 @default.
- W3120602004 cites W2106584820 @default.
- W3120602004 cites W2118329428 @default.
- W3120602004 cites W2123355582 @default.
- W3120602004 cites W2125746373 @default.
- W3120602004 cites W2128274211 @default.
- W3120602004 cites W2131639268 @default.
- W3120602004 cites W2132469484 @default.
- W3120602004 cites W2133682816 @default.
- W3120602004 cites W2134878671 @default.
- W3120602004 cites W2135898863 @default.
- W3120602004 cites W2138709799 @default.
- W3120602004 cites W2140419897 @default.
- W3120602004 cites W2144892629 @default.
- W3120602004 cites W2145842706 @default.
- W3120602004 cites W2146155744 @default.
- W3120602004 cites W2148428508 @default.
- W3120602004 cites W2148968593 @default.
- W3120602004 cites W2149089431 @default.
- W3120602004 cites W2149858388 @default.
- W3120602004 cites W2150246687 @default.
- W3120602004 cites W2236991441 @default.
- W3120602004 cites W2271038606 @default.
- W3120602004 cites W2293067203 @default.
- W3120602004 cites W2320032845 @default.
- W3120602004 cites W2326726820 @default.
- W3120602004 cites W2339658711 @default.
- W3120602004 cites W2463012969 @default.
- W3120602004 cites W2480485646 @default.
- W3120602004 cites W2500218405 @default.
- W3120602004 cites W2511764688 @default.
- W3120602004 cites W2606218280 @default.
- W3120602004 cites W2628145035 @default.
- W3120602004 cites W2742767772 @default.
- W3120602004 cites W2765708136 @default.
- W3120602004 cites W2788733333 @default.
- W3120602004 cites W2794277570 @default.
- W3120602004 cites W2805775386 @default.
- W3120602004 cites W2873011752 @default.
- W3120602004 cites W2892171374 @default.
- W3120602004 cites W2923645205 @default.
- W3120602004 cites W2951168569 @default.
- W3120602004 cites W2982291252 @default.
- W3120602004 cites W2998901800 @default.
- W3120602004 cites W3009972135 @default.