Prognostic Irrelevance of Ring Sideroblast Percentage in World Health Organization Defined Myelodysplastic Syndromes without Excess Blasts,

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 3803-3803
Author(s):  
Mrinal M. Patnaik ◽  
Curtis A Hanson ◽  
Nanna Sulai ◽  
Janice M Hodnefield ◽  
Ryan A Knudson ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Univariate Analysis ◽  
Multivariable Analysis ◽  
Prognostic Scores ◽  
World Health ◽  
Refractory Anemia ◽  
Ring Sideroblasts ◽  
Red Cell Transfusion ◽  
Health Organization ◽  
Risk Categories

Abstract Abstract 3803 Background: Ring sideroblasts (RS) represent abnormal mitochondrial iron deposits that are commonly present in myelodysplastic syndromes (MDS). The presence of ≥15% RS, by definition (World Health Organization-WHO) is necessary for a diagnosis of a MDS-RS; with refractory anemia with ring sideroblasts (RARS) being a specific morphologic category. RS can also be seen with other morphologic categories such as; refractory cytopenias with multilineage dysplasia (RCMD), MDS-unclassifiable (MDS-U) and refractory anemia with excess blasts (RAEB-1/2). Amongst these categories, RARS is generally believed to have the best survival rate with the lowest risk of leukemic transformation and this has been attributed to the absence of associated dysplasia in more than one lineage. However, it has not been systematically shown whether or not the exact percentage of RS provides additional prognostic information in the context of an accurate morphologic categorization and risk stratification by karyotype or the revised IPSS (IPSS-R). Methods: 200 patients with primary MDS without excess blasts and ≥1% RS were seen at the Mayo Clinic from 1997 through 2007. All patients underwent bone marrow (BM) examination and cytogenetic evaluation at diagnosis and the pathology slides, including iron stains, were centrally re-reviewed to accurately quantify bone marrow (BM) RS percentage and to confirm WHO morphologic categories. Molecular profiling included analysis for IDH1, IDH2, JAK2 and MPL mutations. For the purposes of this study patients were divided into 4 categories based on the RS percentage; <5%, 5–14%, 15–50% and >50%. In addition, each patient was assigned IPSS and IPSS-R prognostic scores at diagnosis and risk stratified by karyotype according to the IPSS-R cytogenetic risk categories. Cox proportional regression method was used for multivariable analysis. Results: Of the overall 200 study patients, 140 (70%) were male and median age was 71 years (range, 17–90 years). At presentation, 34 (17%) patients were red cell transfusion-dependent. There were 56 (28%) patients with <5% RS (RCMD-52, MDS-U-14), 32 (16%) with 5–14% RS (RCMD-29, MDS-U-3), 79 (39%) with 15–50% RS (RARS-43, RCMD-29, MDS-U-2) and 33 (16%) with >50% RS (RARS-13, RCMD-17, MDS-U-3). Three patients (2%) were IDH1 mutant (RARS-1, RCMD-2), 17 (8%) IDH2 mutant (RARS-1, RCMD-14, MDS-U-2), 5 JAK2 V617F (3%) mutant (RARS-2, RCMD-3) and one patient with RCMD had the MPL W515L mutation. At a median follow-up of 33 months, 156 (73%) deaths and 24 (12%) leukemic transformations were documented. Median survivals were 63 months for MDS with >50% RS, 43 months for MDS with 15–50% RS, 35 months for MDS with 5–14% RS, and 14 months for MDS with <5% RS (p=0.005). In univariate analysis, additional risk factors included decreased hemoglobin, decreased platelet count, increased circulating blasts, WHO morphologic categories, red cell transfusion need, IPSS, IPSS-R and cytogenetic risk categories per IPSS-R. On a multivariable analysis only the IPSS-R prognostic score (p<0.0001) and the WHO morphologic categories (p=0.02 for RARS) retained significance. Univariate analysis disclosed significantly inferior leukemia-free survival (LFS) in patients with RCMD, thrombocytopenia, increased circulating blasts, and poor IPSS and IPSS-R prognostic scores. On multivariable analysis once again only the IPSS-R prognostic scores (p=0.02) and WHO morphologic categories (p=0.02) retained their significance. In other words, RS percentage, independent of WHO classification, as a continuous or categorical variable (i.e.: <5%, 5–14%, 15–50% & >50%) did not affect either overall survival or LFS. Conclusions: In MDS without excess blasts, once an accurate WHO morphologic categorization is made based on the presence or absence of multi-lineage dysplasia, there is no additional prognostic value for quantifying bone marrow ring sideroblasts. WHO classification and IPSS-R prognostic scores remain the most important factors in assessing patients with MDS. Disclosures: No relevant conflicts of interest to declare.

SF3B1 Mutations Are Prevalent in Myelodysplastic Syndromes with Ring Sideroblasts but Do Not Hold Independent Prognostic Value

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 460-460 ◽  
Author(s):  
Mrinal M. Patnaik ◽  
Terra L Lasho ◽  
Janice M Hodnefield ◽  
Ryan A Knudson ◽  
Rhett P Ketterling ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Myelodysplastic Syndromes ◽  
Prognostic Value ◽  
Univariate Analysis ◽  
Multivariable Analysis ◽  
World Health ◽  
Refractory Anemia ◽  
Ring Sideroblasts ◽  
Risk Categories ◽  
Good Risk

Abstract Abstract 460 Background: SF3B1 mutations were recently reported to occur in myelodysplastic syndromes (MDS), especially in the presence of ring sideroblasts (RS) (Papaemmanuil et al. Leukemia Research 2011, Volume 35, Supplement 1, page S18). We sought to accurately define the interaction between SF3B1 mutations, bone marrow morphology, karyotype and prognosis in MDS with ≥15% RS (MDS-RS). Methods: Mayo Clinic databases and cell banks were queried to identify patients with MDS and ≥15% RS (MDS-RS). All study patients were required to have undergone bone marrow examination and cytogenetic evaluation at diagnosis. Pathology slides, including iron stains, were centrally re-reviewed to accurately quantify bone marrow RS percentage and confirm World Health Organization (WHO) morphologic categories. Detailed analysis of clinical and cytogenetic parameters was performed to risk stratify patients according to the revised International Prognostic Scoring System (IPSS-R) (Greenberg et al. Leukemia Research 2011, Volume 35, Supplement 1, page S6). All patients were annotated for their mutational status involving JAK2, MPL and IDH. PCR sequencing was used to identify SF3B1 mutations. Results I: Baseline patient information: The study is currently ongoing; to date, results are availab1e in 88 patients (median age 72 years, 69% males) including 36 (41%) patients with refractory anemia with RS (RARS), 36 (41%) with refractory cytopenia with multilineage dysplasia and ≥15% RS (RCMD-RS), 11 (12%) with refractory anemia with excess blasts (RAEB)-1 and ≥15% RS (RAEB1-RS) and 5 (6%) with RAEB2-RS. Median (range) values were 9.1 g/dL (5.8–15.7) for hemoglobin, 4.1 × 109/L (1.2–35.2) for leukocytes and 164 × 109/L (6–585) for platelets. IPSS-R risk categories were 20% very good, 49% good, 11% intermediate, 3% poor and 16% very poor. Karyotype was normal in 60%, other very good/good risk 16%, intermediate-risk 2%, and poor/very poor in 22%; 14% had monosomal karyotype (MK). Six (7%), four (5%) and zero (0%) patients displayed IDH, JAK2 or MPL mutations. Red cell transfusion need was documented in 27 (31%) patients. Results II: Prevalence and distribution of SF3B1 mutations and clinical correlates: SF3B1 mutations (all heterozygous) were detected in 40 (∼46%) of all 88 study patients: 25 (63%) K700E, 6 (15%) K666N/Q/R, 3 (8%) E622D, 2 (5%) H662D/Q, 2 (5%) Y623C, 1 (3%) R625C, and 1 (3%) T663I. One patient each displayed both SF3B1K700E and IDH2R140Q or SF3B1K700E and JAK2V617F. SF3B1 mutational frequencies were 69% for RARS, 36% for RCMD-RS, 18% for RAEB1-RS and 0% for RAEB2-RS (p=0.0007). SF3B1 mutations clustered with normal (59%) and other very good/good risk (57%) karyotype and were infrequent in poor/very poor karyotype (5%; p=0.0004). Additional significant correlations were noted between SF3B1 mutations and lower IPSS-R risk category (p=0.0004), advanced age (p=0.006), higher platelet count (p=0.0003). Results II: SF3B1 mutations and prognostic relevance in MDS-RS: To date, 68 (∼77%) deaths and 10 (∼11%) leukemic transformations have been documented. Median follow-up for living patients was 82 months. Univariate survival analysis considering all 88 study patients identified the following as strongly significant risk factors: IPSS-R (p<0.0001), WHO morphologic categories (p<0.0001), cytogenetic risk categories (p<0.0001), and transfusion need (p=0.0003). In multivariable analysis, only IPSS-R and transfusion need remained significant. In univariate analysis, the presence of SF3B1 mutations was significantly associated with better overall (p=0.04) and leukemia-free (p=0.03) survival; however, in both instances, significance was completely accounted for by WHO morphologic risk categorization and the morphology-adjusted p values were 0.6 and 0.2, respectively. In other words, when RARS and RCMD were analyzed separately, there was no additional prognostic value from the presence or absence of SF3B1 mutations (Figures 1 and 2; p=0.67 and 0.44, respectively). Conclusions: SF3B1 mutations are prevalent in RARS and RCMD-RS but do not provide additional, WHO morphology-independent or IPSS-R-independent, prognostic information. Disclosures: No relevant conflicts of interest to declare.

scholarly journals SF3B1 mutations are prevalent in myelodysplastic syndromes with ring sideroblasts but do not hold independent prognostic value

Blood ◽  
2012 ◽  
Vol 119 (2) ◽  
pp. 569-572 ◽  
Author(s):  
Mrinal M. Patnaik ◽  
Terra L. Lasho ◽  
Janice M. Hodnefield ◽  
Ryan A. Knudson ◽  
Rhett P. Ketterling ◽  
...  
Keyword(s):  
World Health Organization ◽  
Myelodysplastic Syndromes ◽  
Prognostic Value ◽  
Univariate Analysis ◽  
World Health ◽  
Refractory Anemia ◽  
Multilineage Dysplasia ◽  
Ring Sideroblasts ◽  
Health Organization ◽  
Risk Categorization

SF3B1 mutations were recently reported in myelodysplastic syndromes (MDSs), especially in the presence of ring sideroblasts (RSs). We sought to define the interaction between SF3B1 mutations, morphology, karyotype, and prognosis in MDS with more than or equal to 15% RS (MDS-RS). We studied 107 patients with MDS-RS, including 48 with refractory anemia with RS (RARS), 43 with refractory cytopenia with multilineage dysplasia (RCMD)-RS, 11 with refractory anemia with excess blasts-1 (RAEB1)–RS, and 5 with RAEB2-RS. SF3B1 mutations were detected in 53 (∼ 50%) patients: 35 RARS (73%), 16 RCMD-RS (37%), and 2 RAEB1-RS (18%). In univariate analysis, the presence of SF3B1 mutations was associated with better overall (P < .01) and leukemia-free (P < .01) survival; however, in both instances, significance was completely accounted for by World Health Organization morphologic risk categorization. In other words, when RARS and RCMD-RS were analyzed separately, there was no additional prognostic value from the presence or absence of SF3B1 mutations.

scholarly journals Prognostic irrelevance of ring sideroblast percentage in World Health Organization–defined myelodysplastic syndromes without excess blasts

Blood ◽  
2012 ◽  
Vol 119 (24) ◽  
pp. 5674-5677 ◽  
Author(s):  
Mrinal M. Patnaik ◽  
Curtis A. Hanson ◽  
Nanna H. Sulai ◽  
Janice M. Hodnefield ◽  
Ryan A. Knudson ◽  
...  
Keyword(s):  
Myelodysplastic Syndromes ◽  
Multivariable Analysis ◽  
World Health ◽  
Refractory Anemia ◽  
Idh Mutations ◽  
Ring Sideroblasts ◽  
Transfusion Dependency ◽  
Health Organization ◽  
The Impact

Abstract The presence of ≥ 15% bone marrow (BM) ring sideroblasts (RS) and < 5% blasts is required for a diagnosis of refractory anemia with ring sideroblasts. We examined the phenotypic and prognostic relevance of this “15%” RS threshold in 200 patients with myelodysplastic syndromes (MDS) without excess blasts and with ≥ 1% RS. The impact of RS% was assessed both as a continuous and categorical variable: < 5% (n = 56), 5%-14% (n = 32), 15%-50% (n = 79), and > 50% (n = 33). RS% correlated (P < .05) directly with age, platelet count, transfusion dependency, BM cellularity, and mutant SF3B1 and inversely with hemoglobin level, multilineage dysplasia, and high-risk karyotype; but did not correlate with IDH mutations. At a median follow-up of 33 months, 156 (73%) deaths and 24 (12%) leukemic transformations were documented. Neither univariate nor multivariable analysis showed significant effect for RS% on overall or leukemia-free survival, suggesting the limited prognostic value of quantifying BM RS in MDS.

ASXL1 Mutations in Myelodysplastic Syndromes with 1% or More Ring Sideroblasts: Prevalence, Clinical Correlates and Prognostic Relevance

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 2882-2882
Author(s):  
Naseema Gangat ◽  
Terra L. Lasho ◽  
Mrinal M Patnaik ◽  
Christy Finke ◽  
Mark R Litzow ◽  
...  
Keyword(s):  
Median Survival ◽  
Myelodysplastic Syndromes ◽  
Univariate Analysis ◽  
Multivariable Analysis ◽  
Refractory Anemia ◽  
Prognostic Impact ◽  
Wild Type ◽  
Monosomy 7 ◽  
Clinical Correlates ◽  
Ring Sideroblasts

Abstract Background: Addition of sex combs-like 1 (ASXL1) is frequently mutated (mutational frequency 14-29%) and is prognostically relevant in myelodysplastic syndromes (MDS) (Bejar, NEJM 2011, Thol, JCO 2011). The prevalence and prognostic impact of ASXL1 mutation in MDS with ring sideroblasts (MDS-RS) is not known. MDS-RS is defined by the presence of ≥15% RS in bone marrow (BM), with refractory anemia with ring sideroblasts (RARS) being the prototype but may also be seen in other categories. Methods : Our institutional database was reviewed to identify patients with WHO-defined primary MDS with ≥1% BM RS. Pathology slides, including iron stains, were reviewed to accurately quantify BM RS percentage and confirm WHO morphologic categories. All patients were annotated for their mutational status including ASXL1, JAK2, MPL and IDH with a subset for SF3B1 by PCR sequencing performed on BM specimens obtained at diagnosis. Results : i) Patient characteristics: A total of 76 MDS patients displayed ≥1% BM RS (median age 72 years; 76% males); 51 (67%) patients had ≥15% BM RS. IPSS-R risk categories for the entire 76 study patients were 30% very low, 37% low, 14% intermediate, 11% high and 8% very high; IPSS-R karyotype was normal in 63%, very good/good risk 17%, intermediate risk 12%, and poor/very poor in 8%; 3% had monosomal karyotype. ii) Prevalence of ASXL1 mutations: Twenty-one (28%) of the 76 study patients were ASXL1 mutated; ASXL1 mutational frequencies were 25% (16/63 patients) in the absence and 38% (5/13 patients) in the presence of excess blasts (P =0.34). When considering only those patients with ≥15% BM RS (n =51), ASXL1 mutations were detected in 12 (24%) patients with mutational frequencies of 24% (11/46 patients) in the absence and 20% (1/5 patients) in the presence of excess blasts (P =0.84); ASXL1 mutational frequencies were 13% (3/23 patients) in RARS and 37% (7/19 patients) in RCMD-RS (P=0.07). In terms of other mutations, all 76 study patients were wild-type for JAK2 and MPL, whereas IDH2 R140Q mutations were present in 4 (5%) patients, including 3 with concomitant ASXL1 and IDH2 mutations. IDH1 mutation was seen in only 1 patient. 25 of 43 (58%) patients screened were mutated for SF3B1, including 4 (9%) who were mutated for both ASXL1 and SF3B1. Significant associations were evident between ASXL1 and IDH2 mutations (P =0.02) but not between ASXL1 and SF3B1 mutations (P =0.61). iii) Clinical correlates of ASXL1 mutations: Among all 76 study patients, presence of ASXL1 mutation did not correlate with age (P =0.30), hemoglobin level (P =0.17), platelet count (P =0.53), BM blast percentage (P =0.17), WHO morphologic category (P =0.34), transfusion dependence (P =0.84), IPSS-R cytogenetic categories (P =0.93), or IPSS-R risk group (P =0.33). The results were unchanged when analyzing the 51 patients with MDS-RS (i.e. ≥15% BM RS). Amongst the ASXL1 mutated patients (n =21) IPSS-R cytogenetic categories were as follows: 13 patients with normal karyotype (62%), 2 patients with trisomy 8 (10%), 1 patient each with -Y, del(11q), del(5q), del(20q), isochromosome 17 and monosomy 7 (5% each). iv) Prognostic impact of ASXL1 mutations: Median follow-up was 42.5 months, during which time 69 (91%) deaths and 8 (11%) leukemic transformations were documented. ASXL1 mutated patients had a median survival of 29 months, compared to 45 months in ASXL1 wild-type patients (P =0.04). However, the difference in median survival was no longer significant during multivariable analysis, which instead identified only IPSS-R and transfusion need as being independent predictors of inferior survival. Amongst those patients without excess blasts (n =63), presence of ASXL1 mutation predicted inferior survival in univariate analysis (P =.04) and significance was sustained during multivariable analysis that included IPSS-R cytogenetic categories (P =.04) but became borderline when WHO morphologic category was included (P =0.06); ASXL1 mutated patients had a shortened median survival of 43 months compared to 66 months in ASXL1 wild-type patients (P =.04). In the presence of ≥15% BM RS, ASXL1 mutations did not affect survival (P =0.48); the results were the same for RARS (P =0.9) and RCMD-RS (P =0.64). Conclusions : ASXL1 mutations might not affect survival in MDS patients with ≥15% BM RS, including those with RARS or RCMD-RS. Furthermore, an apparent survival disadvantage seen in ASXL1 -mutated MDS patients with ≥1% BM RS was accounted for by IPSS-R. Disclosures Pardanani: Stemline: Research Funding.

Systemic mastocytosis in 342 consecutive adults: survival studies and prognostic factors

Blood ◽  
2009 ◽  
Vol 113 (23) ◽  
pp. 5727-5736 ◽  
Author(s):  
Ken-Hong Lim ◽  
Ayalew Tefferi ◽  
Terra L. Lasho ◽  
Christy Finke ◽  
Mrinal Patnaik ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Mast Cell ◽  
Prognostic Factors ◽  
Systemic Mastocytosis ◽  
Multivariable Analysis ◽  
Cell Lineage ◽  
World Health ◽  
Additional Information ◽  
Survival Studies ◽  
Health Organization

Abstract Clinical phenotype in systemic mastocytosis (SM) is markedly variable, which complicates prognostication and decision making regarding the choice and timing of therapy. In a retrospective study of 342 consecutive adult patients with SM seen at the Mayo Clinic between 1976 and 2007, disease subdesignation according to the World Health Organization (WHO) proposal was indolent (ISM) in 159 (46%), with associated clonal hematologic non–mast cell lineage disease (SM-AHNMD) in 138 (40%), aggressive (ASM) in 41 (12%), and mast cell leukemia in 4 (1%). KITD816V was detected in bone marrow–derived DNA by allele-specific polymerase chain reaction (PCR) in 68% of 165 patients evaluated (ISM, 78%; ASM, 82%; SM-AHNMD, 60%; P = .03); JAK2V617F was detected in 4%, all in SM-AHNMD. Compared with those with nonindolent SM, life expectancy in ISM was superior and not significantly different from that of the age- and sex-matched US population. In addition, multivariable analysis identified advanced age, weight loss, anemia, thrombocytopenia, hypoalbuminemia, and excess bone marrow blasts as independent adverse prognostic factors for survival. The current study validates the prognostic relevance of the WHO subclassification of SM and provides additional information of value in terms of both risk stratification and interpretation of clinical presentation and laboratory results.

Difference in clinical features between Japanese and German patients with refractory anemia in myelodysplastic syndromes

Blood ◽  
2005 ◽  
Vol 106 (8) ◽  
pp. 2633-2640 ◽  
Author(s):  
Akira Matsuda ◽  
Ulrich Germing ◽  
Itsuro Jinnai ◽  
Motohiro Misumi ◽  
Andrea Kuendgen ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Clinical Features ◽  
Cumulative Risk ◽  
Japanese Patients ◽  
World Health ◽  
Refractory Anemia ◽  
Ringed Sideroblasts ◽  
Fab Classification ◽  
Health Organization ◽  
French American British

AbstractSeveral reports indicate that there might be differences in clinical features between Asian and Western myelodysplastic syndrome (MDS) cases. We analyzed refractory anemia (RA) in French-American-British (FAB) classification cases diagnosed in Japan and Germany to perform a more exact comparison between Asian and Western MDS types. In the first step, we analyzed agreement of morphologic diagnosis between Japanese and German hematologists. Blood and bone marrow slides of 129 patients diagnosed with FAB-RA, FAB-RA with ringed sideroblasts (RARS), or aplastic anemia were selected randomly and evaluated separately by each group. The agreements of diagnoses according to FAB and World Health Organization (WHO) classifications were 98.4% and 83.8%, respectively. Second, we compared clinical features between 131 Japanese and 597 German patients with FAB-RA. Japanese patients were significantly younger than German patients. Japanese patients had more severe cytopenias. However, prognosis of Japanese patients was significantly more favorable than that of German patients. Japanese patients had a significantly lower cumulative risk of acute leukemia evolution than did German patients. Frequency of WHO-RA in Japanese patients with FAB-RA was significantly higher than that in German patients. In conclusion, our results indicate that the clinical features of Japanese patients with FAB-RA differ from those of German patients.

SF3B1 Mutations Are Associated with Favorable Cytogenetics in Patients with Myelodysplastic Syndromes with Ring Sideroblasts

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 3823-3823 ◽  
Author(s):  
Vincent-Philippe Lavallée ◽  
David Faucher ◽  
Bruno Lamontagne ◽  
Josée Hébert ◽  
Lambert Busque
Keyword(s):  
Bone Marrow ◽  
Myelodysplastic Syndromes ◽  
World Health ◽  
Refractory Anemia ◽  
Wild Type ◽  
Event Free Survival ◽  
Free Survival ◽  
Mutational Hotspots ◽  
Ring Sideroblasts ◽  
Bone Marrow Blasts

Abstract Abstract 3823 Background: Somatic mutations of splicing factor 3B, subunit 1 (SF3B1) are prevalent in myelodysplastic syndromes (MDS) with ring sideroblasts (RS). Little is known about the clinical phenotype associated with these mutations. Initial studies have reported an excellent event-free survival (EFS) in patients with SF3B1 mutations but subsequent reports did not confirm that observation. Therefore, its clinical significance remains uncertain. Our objective was to determine if the SF3B1 mutational status predicts distinctive morphological, cytogenetic or clinical characteristics in patients with MDS with RS. Methods and patients: Twenty-seven patients diagnosed with MDS and ≥ 15% RS in bone marrow aspiration between 2000 and 2011 at our institution and other hospitals affiliated to the Quebec Leukemia Cell Bank (BCLQ) and with available material for genetic analyses were included in this study. Morphology of samples was revised. After DNA extraction, polymerase chain reaction was performed to amplify SF3B1 mutational hotspots in exons 13 to 15. Amplicons were sequenced by the Sanger method. Clinical and laboratory data were collected retrospectively from hospital medical records and BCLQ database. Results: Point mutations of SF3B1 were found in 48% (13/27) with the following distribution: refractory anemia with RS (RARS): 7/10, refractory cytopenia with multilineage dysplasia (RCMD): 5/10 and refractory anemia with excess blasts (RAEB): 1/7. All mutations were heterozygous nonsense mutations and K700E exon 15 mutations accounted for 85% (11/13) of mutations. The remaining mutations were located in exon 14 at position 666. In the entire cohort, SF3B1 mutations were associated with an older median age at diagnosis (69 vs 60 years, p= 0.007). They were associated with favorable cytogenetics whereas unmutated SF3B1 was associated with unfavorable cytogenetics (8 cases vs 0 with favorable cytogenetics and 0 cases vs 11 with unfavorable cytogenetics for mutated and wild type SF3B1 respectively, p < 0.0001). Median overall survival (OS) was similar in the 2 groups (not reached vs 27 months, p=0.7) but median EFS was higher in patients with mutated SF3B1 (not reached vs 5 months, p=0.02). To evaluate the influence of higher bone marrow blasts on outcomes, analyses were restricted to the RARS and RCMD-RS categories. In that subgroup, SF3B1 mutations were correlated with higher median platelet counts at diagnosis (201 vs 70 × 109/L, p=0,04). In addition, SF3B1 mutations remained associated with favorable cytogenetics, and unmutated SF3B1 with unfavorable cytogenetics (p=0.004). Moreover, SF3B1 mutations were more frequently detected within favorable or intermediate-1 categories of the International Prognostic Scoring System (IPSS) compared to wild type SF3B1 (p=0.04). However, in patients with < 5% bone marrow blasts, EFS was similar between mutated and unmutated SF3B1 (not reached vs 46 months, p =0.4). Conclusion: In this series, SF3B1 mutations are frequent in MDS with RS and they are strongly associated with favorable cytogenetics. They are mostly detected among low-risk groups of the World Health Organisation classification (RARS and RCMD-RS), but they are not independently associated with an increased overall or event-free survival. Disclosures: No relevant conflicts of interest to declare.

Azatidine Treatment in Very Elderly Patients Diagnosed with Acute Myelogenous Leukemia (AML) or High Risk Myelodysplastic Syndrome (MDS)

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 5262-5262
Author(s):  
Javier Gutierrez ◽  
Elena Morales ◽  
Matilde Gonzalez ◽  
Juan Herrada
Keyword(s):  
Bone Marrow ◽  
High Risk ◽  
Conflicts Of Interest ◽  
Myelogenous Leukemia ◽  
World Health ◽  
Refractory Anemia ◽  
Very Elderly ◽  
Elderly Age ◽  
Multilineage Dysplasia ◽  
Health Organization

Abstract Background: The use of azatidine (AZT) is the agent of choice for first line treatment of elderly (age >74 years) patients (pts) with high risk MDS or AML (2015 National Comprehensive Cancer Network Guidelines). The Hematology consultant is frequently involved in the care of very elderly (age >79 years) individuals. Our aim is to report our experience with 3 patient aged 80 or above that received treatment with AZT. Methods: Retrospective review Results: We reviewed the medical records of patients treated for high risk MDS or AML at our institution between July 2009 and March 2012. A total of 3 pts aged 80 or above were identified. All three pts were males, and all received AZT 100 mg/m2/day x 7 days subcutaneously in every cycle of 28 days. Patient 1 (aged 81) had 12 % of blasts in the bone marrow (BM), was diagnosed with Refractory Anemia with Excess Blasts-2 (RAEB-2) applying the 2008 World Health Organization Classification (WHO) of MDS. Patient 2 (aged 90) had AML with multilineage dysplasia and 38% blasts in BM. Patient 3 (aged 80) had AML with multilineage dysplasia and 30% blasts in BM. Pts characteristics and results are summarized in Table 1. Conclusions: In pts diagnosed with high risk MDS or AML, advanced chronological age (> 79 years) alone should not preclude the use of effective and well tolerated therapy. Identification of pts in this geriatric subgroup who may benefit from AZT merits additional research. Table 1. Diagnosis Age(years) Baseline laboratory Peripheral blood %Blast in Bone Marrow Number of AZT cycles received Response Survival(months) Patient 1 RAEB-2 81 Hb 14 g/dl Platelets 56x10e9/L Leucocytes 1.90x10e9/L (Neutrophils 0.81x10e9/L) 12 11 CR* in BM after 6 cycles 17 Patient 2 AML 90 Hb 8.06 g/dl Platelets 20x10e9/L Leucocytes 1.30x10e9/L(Neutrophils 0.64x10e9/L) 38 15 CR in BM after 7 cycles 22 Patient 3 AML 80 Hb 7.50 g/dl Platelets 30x10e9/L Leucocytes 6.00x10e9/L(Neutrophils 1.44x10e9/L) 30 19 CR in BM after 6 cycles 23 *CR: complete remission (<5% blast in aspirate with spicules) Disclosures No relevant conflicts of interest to declare.

HUBUNGAN ANTARA PENDIDIKAN DAN PEKERJAAN IBU HAMIL DENGAN PEMERIKSAAN KEHAMILAN DI PUSKESMAS MARIANA KECAMATAN BANYUASIN I KABUPATEN BANYUASIN TAHUN 2011

2019 ◽  
Vol 2 (1) ◽  
Author(s):  
Leny Leny
Keyword(s):  
Prenatal Care ◽  
Pregnant Women ◽  
Maternal Education ◽  
Statistical Tests ◽  
Univariate Analysis ◽  
World Health ◽  
P Value ◽  
Chi Square ◽  
Cross Sectional ◽  
Health Organization

ABSTRACT Prenatal care is health care by health personnel to care the pregnant according to standards. Worlrd Health Organization (WHO) estimates more than 500.000 women die during pregnancy or childbirth. Maternal mortality in Indonesia is 307 per 100,000 live births. The quantity of pregnant women’s visit in Kabupaten Banyuasin in 2009 of 89.1%. The purpose of this study to determine the relationship between education and occupation with prenatal care at Puskesmas Mariana  Kecamatan Banyuasin I Kabupaten Banyuasin in 2011. This study uses analytic approach survey by Cross Sectional methods, the population are 1.946 pregnant women and the samples as many as 332 people. The results of univariate analysis study of pregnant women who are higher education as much as 45.2%, and  low maternal education as much as 54.8%. In pregnant women who work of 43.4%, and pregnant women who do not work for 56.6%. From the results of bivariate analysis and Chi-Square statistical tests found a significant association between education of pregnant women with prenatal care with P Value = 0.000, and there was a significant association between occupation of pregnant women with prenatal care with P Value = 0.000. Can be concluded that there is a relationship between education and occupation of pregnant women with prenatal care. Expected to health workers to provide counseling on the importance of prenatal care in pregnant women and expected future studies may explore again the factors associated with prenatal care with the different variables.   ABSTRAK Pemeriksaan kehamilan adalah pelayanan kesehatan oleh tenaga kesehatan untuk memeriksakan ibu hamil sesuai standar. World Health Organization (WHO) memperkirakan lebih dari 500.000 ibu pertahunnya meninggal saat hamil atau bersalin. AKI di Indonesia 307 per 100.000 kelahiran hidup. Jumlah kunjungan ibu hamil di Kabupaten Banyuasin tahun 2009 sebesar 89,1%. Tujuan penelitian ini untuk mengetahui hubungan antara pendidikan dan pekerjaan dengan pemeriksaan kehamilan di Puskesmas Mariana Kecamatan Banyuasin I Kabupaten Banyuasin tahun  2011. Penelitian ini menggunakan metode survey analitik dengan pendekatan Cross Sectional, populasi ibu hamil dengan jumlah 1.946 orang dan jumlah sampel sebanyak 332 orang. Hasil penelitian Analisa Univariat adalah ibu hamil yang pendidikan tinggi sebanyak 45,2%, dan pendidikan rendah ibu hamil sebanyak 54,8%. Pada variabel pekerjaan ibu hamil yang bekerja sebesar 43,4%, dan ibu hamil yang tidak bekerja sebesar 56,6%. Dari hasil analisa bivariat dan uji statistik Chi-Square  didapatkan hubungan yang bermakna antara pendidikan ibu hamil dengan pemeriksaan kehamilan dengan  P Value = 0,000, dan ada hubungan yang bermakna antara pekerjaan ibu hamil dengan pemeriksaan kehamilan dengan P Value = 0,000. Dapat disimpulkan bahwa ada hubungan antara pendidikan dan pekerjaan ibu hamil dengan pemeriksaan kehamilan. Diharapkan kepada petugas kesehatan agar dapat memberikan penyuluhan tentang pentingnya pemeriksaan pada ibu hamil dan diharapkan penelitian yang akan datang dapat menggali lagi faktor-faktor yang berhubungan dengan pemeriksaan kehamilan dengan variabel yang berbeda.

scholarly journals A review of current knowledge of myeloproliferative disorders in the horse

2021 ◽  
Vol 63 (1) ◽  
Author(s):  
Katy Satué ◽  
Juan Carlos Gardon ◽  
Ana Muñoz
Keyword(s):  
Bone Marrow ◽  
Myeloid Leukemia ◽  
Current Knowledge ◽  
Myeloproliferative Neoplasms ◽  
Myeloproliferative Disorders ◽  
World Health ◽  
Chronic Granulocytic Leukemia ◽  
Current State ◽  
Multiple Cell ◽  
Health Organization

AbstractMyeloid disorders are conditions being characterized by abnormal proliferation and development of myeloid lineage including granulocytes (neutrophils, eosinophils and basophils), monocytes, erythroids, and megakaryocytes precursor cells. Myeloid leukemia, based on clinical presentation and proliferative rate of neoplastic cells, is divided into acute (AML) and myeloproliferative neoplasms (MPN). The most commonly myeloid leukemia reported in horses are AML-M4 (myelomonocytic) and AML-M5 (monocytic). Isolated cases of AML-M6B (acute erythroid leukemia), and chronic granulocytic leukemia have also been reported. Additionally, bone marrow disorders with dysplastic alterations and ineffective hematopoiesis affecting single or multiple cell lineages or myelodysplastic diseases (MDS), have also been reported in horses. MDSs have increased myeloblasts numbers in blood or bone marrow, although less than 20%, which is the minimum level required for diagnosis of AML. This review performed a detailed description of the current state of knowlegde of the myeloproliferative disorders in horses following the criteria established by the World Health Organization.

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