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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Recent studies of acute erythroleukemias have reaffirmed DiGuglielmo's syndrome (M6a, myeloblast-predominant) and disease (M6b, pronormoblast-predominant). M6c (mixed myeloblast/pronormoblast) has also been described. However, MDS is still defined according to the percentage of myeloblasts (% myeloblasts) without including the pronormoblast count. A 20-year retrospective study was performed to identify cases demonstrating >or=50% erythrocytic component and <30% calculated blasts (FAB exclusion criteria) without underlying cause (96 cases). Pronormoblast and myeloblast counts and other variables were analyzed as possible explanatory variables of the variations in survival. Considered alone, increasing % myeloblasts and/or percentage of pronormoblasts (% pronormoblasts) were significant predictors of decreasing survival. When all variables were considered as a multivariate group, the best fitting statistical model for predicting survival was a function of age, % pronormoblasts, IPSS cytopenias, platelet count, and percentage erythrocytic component. Of these, % pronormoblasts was by far the most significant. Nonappearance of % myeloblasts in this model is indicative of high correlations of this count with other variables.
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PMID:Prognostic significance of pronormoblasts in erythrocyte predominant myelodysplastic patients. 1675 68

In the FAB (French-American-British) and WHO (World Heath Organization) classifications, the blasts in erythroleukemia (M6a) are enumerated from the marrow nonerythroid rather than the total-nucleated cells. However, the method for blast calculation in erythroid-predominant myelodysplastic syndrome (erythroblasts>or=50%) is not specified either in the FAB or WHO classifications. We retrieved the files of 74 erythroid-predominant myelodysplastic syndrome patients (17% of all myelodysplastic syndrome) and 192 myelodysplastic syndrome controls (erythroblasts<50%). In erythroid-predominant myelodysplastic syndrome, by enumerating blasts from marrow nonerythroid cells rather than from total nucleated cells, 41 of 74 (55%) cases would be upgraded, either by disease subcategory or International Prognostic Scoring System. Importantly, the patients with <5% blasts demonstrated a superior survival to patients with >or=5% blasts (P=0.002); this distinction was lost when blasts were calculated from total-nucleated cells. Of cases with >or=5% blasts, cytogenetics rather than blast count correlated with survival. We conclude that in erythroid-predominant myelodysplastic syndrome, blast calculation as a proportion of marrow nonerythroid rather than total nucleated cells can better stratify patients into prognostically relevant groups.
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PMID:Erythroid-predominant myelodysplastic syndromes: enumeration of blasts from nonerythroid rather than total marrow cells provides superior risk stratification. 1883 18