scholarly journals Marrow Blast Percentage Impact on High-Grade Myelodysplastic Syndrome By the Revised International Prognostic Scoring System

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5510-5510
Author(s):  
Omar Alkharabsheh ◽  
Mrinal M. Patnaik ◽  
Naseema Gangat ◽  
Kebede H. Begna ◽  
Hassan B. Alkhateeb ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Overall Survival ◽  
Myelodysplastic Syndrome ◽  
Scoring System ◽  
International Prognostic Scoring System ◽  
High Grade ◽  
Survival Difference ◽  
Significant Difference ◽  
Risk Of Progression ◽  
Very High

Abstract Introduction: The revised international prognostic scoring system (IPSS-R) for myelodysplastic syndrome (MDS) is widely accepted and has been validated in multiple studies. Patients with adverse cytogenetics do poorly and that is reflected in this scoring system by having the highest score for cytogenetics; 2 for intermediate, 3 for poor and 4 for very poor. Little is known about the effect of marrow blasts in adverse cytogenetic in the high-grade MDS defined by IPSS-R intermediate (>3), high (>4.5) and very high (>5). The goal is to examine the effect of marrow blast percentage on outcome in patients with adverse cytogenetics that is present in the high-grade MDS. Methods: We performed data collection from the Mayo clinic records for patients with confirmed MDS after obtaining appropriate IRB approval. Patients were divided based on their total IPSS-R score and we extracted high-grade MDS cases with intermediate, high and very-high IPSS-R only. Cytogenetics and baseline CBC were available for analysis. We calculated the survival difference in patients with blasts <5% and patient with blasts of 5% to 19% for every group. Survival estimates were calculated by Kaplan-Meier method and compared by log-rank testing using JMP v.13. Results: Our database had 1300 patients with confirmed MDS, 41% (N=536) are high-grade MDS. From those, the median age was 70 and 70% were males. Median bone marrow blast was 6% (0-19). Baseline hemoglobin is 9.2 g/dL, WBC 2.7, ANC 1.05, and platelets 69. Their cytogenetics were 1% very good, 31% good, 23% intermediate, 16% poor and 29% very poor. The total IPSS-R groups were 39%,31%, and 30% for the intermediate, high, and very-high groups respectively. The overall survival (OS) for the high-grade MDS with marrow blast <5% was 12.3 months and for patients with marrow blasts ≥5% 11.4 months (P=.4). At each high-grade MDS; intermediate, high and very high, there were no statistically significant differences for patients with marrow blasts below or above 5%. In term of risk of progression to AML, patients with blasts ≥5% were at higher risk of progression compared with <5% (25% vs 10% , P<.001), with no statistically significant difference in term of time-to-AML progression. Conclusion: The percentages of bone marrow blasts had no impact on overall survival among patients with high grade MDS. However, patients with ≥5% marrow blasts are at a higher risk for progression to AML. Disclosures Al-Kali: Novartis: Research Funding.

scholarly journals CIRCULATING microRNA EXPRESSION IN MYELODYSPLASTIC SYNDROME

2019 ◽  
Vol 141 (7-8) ◽  
pp. 233-237
Keyword(s):  
Bone Marrow ◽  
Myelodysplastic Syndrome ◽  
Peripheral Blood ◽  
Scoring System ◽  
Molecular Mechanisms ◽  
International Prognostic Scoring System ◽  
Circulating Microrna ◽  
Hematopoietic Stem ◽  
The Impact

Myelodysplastic syndrome (MDS) is a clonal hematopoietic stem cell disorder characterized by ineffective hematopoiesis and cytopenia in peripheral blood, where about a third of patients may develop acute myeloid leukemia (AML). The diagnosis of MDS requires the analysis of peripheral blood and bone marrow. Depending on the percentage of blasts in the bone marrow, the number of cytopenias and cytogenetic abnormalities, determination of the prognostic indices is possible (IPSS – „International Prognostic Scoring System“, R-IPSS-„Revised International Prognostic Scoring System“, WPSS – „WHO Prognostic Scoring System“). Until today, numerous studies have been conducted on the molecular mechanisms and epigenetic pathways in myelodysplastic syndrome, and their prognostic and therapeutic importance, but there are few studies analyzing the importance of microRNAs (miRNAs) in MDS. In the last few years, there have been numerous results on the impact of aberrant miRNA expression in malignant disorders where the miRNA represent tumor suppressor genes or oncogenes. Several miRNAs have been recognized as diagnostic and prognostic parameters and possible therapeutic targets. In this paper, we present the overview of recent results on the role of miRNA in MDS.

Discrepancy of blast percentage between the bone marrow aspirate and biopsy and its impact on survival outcomes in patients with myelodysplastic syndromes excess blast (MDS-EB).

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. 7053-7053
Author(s):  
Meera Yogarajah ◽  
Phuong L. Nguyen ◽  
Rong He ◽  
Hassan Alkhateeb ◽  
Mithun Vinod Shah ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Scoring System ◽  
Small Sample Size ◽  
Scoring Systems ◽  
Standard Of Care ◽  
Small Sample ◽  
International Prognostic Scoring System ◽  
Increased Risk ◽  
Significant Difference ◽  
Impact On Survival

7053 Background: The revised International Prognostic Scoring System (IPSS-R) aids in prognosticating MDS. The percentage (%) of blasts in the bone marrow is one of the major determinants of the scoring system. The aspirate blast % is utilized as the standard of care, but there could be discrepancies in the blast % reported by the aspirate and the biopsy. We aim to study the possible use of bone marrow biopsy blasts in MDS-EB in calculating IPSS-R. Methods: The MDS database was reviewed for cases of MDS-EB after due IRB approval. We calculated IPSS-R scores based on the aspirate blast % (IPSS-RAsp) and biopsy blast % (IPSS-RBx). The biopsy blast % was reported morphologically or by the CD34 stain. Whenever a range was reported the highest value was utilized as the blast %. Suboptimal aspirates were excluded from the study. The overall survival (OS) was determined by IPSS-RAsp, IPSS-RBx and IPSS-R highest blast (IPSS-RHi). OS estimates were calculated by Kaplan-Meier curves and log-rank testing using JMP v.13. Uno’s concordance statistic was used to compare all 3 risk scoring systems. Results: Of 1322 patients, 431 (33%) cases were identified with MDS-EB; out of which 173 cases had both blasts reported in the biopsy and the aspirate. Out of 173 cases, 35 (20%) had MDS-EB1, and 61 (35%) had MDS EB-2 based on both biopsy and aspirate (concordant cases). Seventy seven (45%) patients changed from EB-1 to EB2 or vice versa based on the biopsy blast (44/77 (57%) cases were upstaged). The OS outcomes based on the IPSS-RBx biopsy showed a clear and meaningful separation with median OS decreasing with increased risk but IPSS-RAsp and IPSS-RHi did not (Table). We compared the 3 models for observed OS differences using the Uno model and there was no statistically significant difference. Conclusions: IPSS-RBx (but not IPSS-RAsp and IPSS-RHi) identified prognostic groups for OS with median OS decreasing with increased risk. The small sample size may have led to an insignificant effect on model power by Uno model. This finding needs to be validated by other centers. [Table: see text]

scholarly journals Overall Survival in Patients with Myelofibrosis Who Have Discontinued Ruxolitinib: A Literature Review

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 3457-3457
Author(s):  
Derek Tang ◽  
Ankush Taneja ◽  
Preety Rajora ◽  
Indeg Sly ◽  
Niall James Davison
Keyword(s):  
Bone Marrow ◽  
Overall Survival ◽  
High Risk ◽  
Literature Review ◽  
Survival Data ◽  
Scoring System ◽  
Janus Kinase ◽  
Cochrane Library ◽  
International Prognostic Scoring System ◽  
Independent Reviewer

Introduction: Myelofibrosis (MF) is a rare bone marrow cancer characterized by bone marrow fibrosis and abnormal cytokine expression, often leading to splenomegaly, constitutional symptoms, and cytopenia. Prognostic scoring systems (the International Prognostic Scoring System and the Dynamic International Prognostic Scoring System) classify patients into low-, intermediate-1-, intermediate-2-, or high-risk categories. Most patients with MF have either intermediate-2- or high-risk disease, indicating a poor overall prognosis and short survival time (5-year survival rate in Europe: 35%). Ruxolitinib is the first Janus kinase inhibitor (JAKi) treatment for MF approved by the US Food and Drug Administration and the European Medicines Agency. However, the rate of discontinuation of ruxolitinib in the first 2-3 years of treatment is high (&gt; 50%) due to treatment resistance, disease recurrence, and worsening of anemia. This literature review aimed to assess overall survival (OS) in patients with MF who have discontinued ruxolitinib. Methods: A systematic literature review (SLR) was conducted in which Embase®, MEDLINE®, and the Cochrane Library were searched to identify published evidence from database inception until August 2018. Conference proceedings, health technology assessments, and bibliographies were also searched. Additionally, this SLR was updated in a targeted manner using Embase® until February 2019 to identify and update the OS evidence among patients with MF who have discontinued ruxolitinib. Retrieved studies were included if they were published in English and reported OS data in the targeted patient population of interest. Two independent reviewers assessed the studies against pre-defined eligibility criteria (Table 1) to include or exclude the studies, and any uncertainty was resolved by a third independent reviewer, in the case of the SLR, or by mutual agreement, in the case of the update. All extracted data were quality checked by a second independent reviewer. A descriptive, qualitative analysis was conducted to assess OS in patients with MF who have used and discontinued ruxolitinib. Results: Of the 4,011 publications retrieved, 11 studies were included (Table 2). Six were retrospective observational studies, 2 were randomized controlled trials (RCTs), and 3 were non-RCTs. Across all the included studies, 5 reported estimates of median OS. Across the 4 studies reporting median OS for standard of care or approved treatments, median OS ranged from 4.9-30 months (Kuykendall et al. 2017, Mehra et al. 2016, Newberry et al. 2017, Palandri et al. 2018). Patients receiving no treatment after ruxolitinib had a median OS of 4.9 months (Kuykendall et al., 2017). Median OS in patients who received treatment with salvage therapy or conventional agents (e.g. hydroxyurea, danazol, anagrelide) was typically around 14 months (14, 14, and 15 months in Mehra et al. 2016, Newberry et al. 2017, and Kuykendall et al. 2017, respectively). Estimated median OS following ruxolitinib discontinuation for early discontinuers and spleen responders in the COMFORT-II study was approximately 16.5 months (NICE, 2015). One study reported median OS for an investigational agent (Mascarenhas et al. 2018); median OS was 19.9 and 29.9 months for imetelstat 4.7 mg/kg and 9.4 mg/kg, respectively. Conclusions: This literature review revealed that patients with MF generally experience poor OS after discontinuing ruxolitinib, especially in patients who receive no further treatments. Line of therapy definitions were rarely reported across studies, which may contribute to variations across study findings. In addition, survival estimates after prior ruxolitinib therapy varied depending on the treatment received and the reason for discontinuation of ruxolitinib. Limited survival data for investigational therapies were available from early-stage trials and may be subject to substantial variations in large-scale registrational trials. Some of the studies included in this literature review may be ongoing as they are currently available in abstract form only, and new data may become available in the near future. Sustained efforts to develop more effective treatments for patients with MF who have discontinued ruxolitinib are imminently needed. Disclosures Tang: Celgene Corporation: Employment, Equity Ownership. Taneja:BresMed Health Solutions Ltd: Employment. Rajora:BresMed Health Solutions Ltd: Employment. Sly:BresMed Health Solutions Ltd: Employment. Davison:BresMed Health Solutions Ltd: Employment.

Colony-Forming Unit Cell (CFU-C) Assays in Myelodysplastic Syndrome

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 2874-2874
Author(s):  
Bing Li ◽  
Jinqin Liu ◽  
Shiqiang Qu ◽  
Robert Peter Gale ◽  
Ruixian Xing ◽  
...  
Keyword(s):  
Overall Survival ◽  
Scoring System ◽  
Conflicts Of Interest ◽  
Unit Cell ◽  
International Prognostic Scoring System ◽  
Hematopoietic Stem ◽  
Colony Forming Unit ◽  
Significant Difference

Abstract Introduction: The myelodysplastic syndromes (MDS) are a group of clonal diseases derived from hematopoietic stem cells (HSC). Colony-forming unit cell (CFU-C) assay is an effective method to study the number and the function of HSC in vitro. In this study, we focus on the characteristics and the prognostic value of CFU-C in patients with MDS. Patients and Method: CFU-C assays were performed according to the protocol of MethoCultTM H4435 Enriched (STEMCELL Technologies). A colony was defined as an aggregate of >40 cells. Clusters consisted of 4 to 40 cells. 560 consecutive newly-diagnosed, untreated subjects with MDS diagnosed from March, 2001 to April, 2013 were studied. All subjects were reclassified according to the 2008 WHO criteria. 535 subjects with evaluable cytogenetics were classified using the International Prognostic Scoring System (IPSS) and the revised International Prognostic Scoring System (IPSS-R) criteria. Follow-up data were available for 470£¨84%£©subjects. Median follow-up of survivors was 26 months (range, 1-170) months. Subjects receiving an allotransplants were censored in survival analyses. Erythroid and myeloid colonies were isolated from each subject with one cytogenetic abnormality such as del(5/5q-) or +8. Cytogenetic abnormalities of each colony were analyzed by fluorescence in situ hybridization (FISH). SPSS 17.0 software was used to make statistical analysis. Results: Frequencies of burst-forming units-erythroid (BFU-E), colony forming unit-erythroid (CFU-E) and colony forming unit-granulocytes/macrophages (CFU-G/M) were significantly lower than normals (P<0.05) (Table 1). Subjects classified as lower risk in IPSS and IPSS-R had significantly higher numbers of BFU-E and CFU-E (P<0.05) but similar numbers CFU-G/M and clusters-G/M compared with higher risk subjects (Table 2). In 11 subjects with del(-5/5q-) or +8 identified by G- and/or R-banding, both normal and abnormal CFU-Cs were identified in 8 subjects studied by FISH. A high ratio of cluster- to CFU-G/M (>0.6) was associated with poor-risk cytogenetics (Table 2) and with worse overall survival in univariable (Figure 1, P=0.001) and multivariable analyses (HR 1.748, [1.01-3.0]; P=0.046) after adjusting for IPSS. Conclusions: These data suggest abnormalities of proliferation and differentiation of erythroid and myeloid precursor cells in vitro parallel the ineffective hematopoiesis typical of MDS and may be useful in predicting outcomes of patients with MDS. Table 1. CFU-C in MDS subtypes N BFU-E CFU-E CFU-G/M N Ratio of cluster- to CFU-G/M RA 21 8 (0-44) 40 (0-134) 14 (0-127)1 6 0.25 (0.40-1.00) RT 4 18 (4-55) 75 (60-90)1 30 (18-70)1 2 2 RARS 27 12 (0-33) 35 (1-140) 12 (0-70)1 10 0.45 (0.17-0.80) RCMD 275 10 (0-80) 33 (0-178) 14 (0-100) 126 0.35 (0-0.83) RAEB1 112 10 (0-258) 32 (0-312) 14 (0-89) 53 0.47 (0-1.00) RAEB2 103 9 (0-46) 25 (0-120) 13 (0-72) 42 0.37 (0-1.00) MDS-U 15 4 (0-58) 25 (0-161) 10 (0-43) 3 2 Del(5q) 3 2 (2-4) 15 (0-20) 5 (5-41)1 1 2 1No significant difference compared with normals. 2Too few cases to analyze. Table 2. Associations between CFU-C and clinical and laboratory variables N BFU-E P CFU-E P CFU-G/M P Number Ratio of cluster- to CFU-GM P IPSS 0.064 0.006 0.361 0.089 Low 30 13 (0-44) 60 (0-169) 19 (0-45) 10 0.44 (0.24-0.70) Int-1 361 10 (0-258) 33 (0-312) 14 (0-127) 150 0.33 (0-1.00) Int-2 115 9 (0-61) 30 (0-137) 14 (0-72) 52 0.45 (0-1.00) High 29 7 (0-34) 21 (0-93) 12 (0-67) 12 0.44 (0-1.00) IPSS-R 0.003 0.003 0.125 0.209 Very low 7 16 (9-25) 30 (15-120) 18 (5-33) 2 0.29 (0.10-0.49) Low 130 14 (0-80) 42 (0-178) 17 (0-70) 48 0.31 (0-0.77) Intermediate 173 10 (0-66) 34 (0-161) 13 (0-127) 81 0.37 (0-1.00) High 139 9 (0-259) 29 (0-312) 11 (0-89) 51 0.33 (0-1.00) Very high 86 8 (0-61) 25 (0-137) 14 (0-91) 42 0.47 (0-1.00) Cytogenetics (IPSS) 0.867 0.055 0.290 0.007 Good 327 10 (0-258) 36 (0-312) 15 (0-89) 133 0.33 (0-1.00) Intermediate 133 10 (0-69) 30 (0-162) 12 (0-127) 63 0.45 (0-1.00) Poor 75 10 (0-61) 25 (0-137) 14 (0-91) 28 0.42 (0-1.00) Cytogenetics (IPSS-R) 0.990 0.090 0.676 0.022 Very good 7 11 (4-20) 48 (1-110) 14 (8-28) 2 0.49 (0.43-0.56) Good 324 10 (0-258) 35 (0-312) 15 (0-89) 132 0.33 (0-1.00) Intermediate 129 10 (0-69) 30 (0-162) 12 (0-127) 62 0.45 (0-1.00) Poor 27 10 (0-61) 35 (0-137) 16 (0-48) 8 0.36 (0.15-1.00) Very poor 48 11 (0-42) 22 (0-120) 14 (0-91) 20 0.53 (0-1.00) Figure 1. Overall survival in subjects with cluster- to CFU-G/M ratios ¡Ü or > 60%. Figure 1. Overall survival in subjects with cluster- to CFU-G/M ratios ¡Ü or > 60%. Disclosures No relevant conflicts of interest to declare.

Outcomes of Hematopoietic Stem Cell Transplant in Older Patients with Myelodysplastic Syndrome and Co-Existing Myelofibrosis: A Single Center Experience and Review of the Literature

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 5850-5850
Author(s):  
Abhijit B Saste ◽  
Philip Kuriakose ◽  
Nalini Janakiraman ◽  
Edward Peres ◽  
Shatha Y. Farhan
Keyword(s):  
Bone Marrow ◽  
Overall Survival ◽  
Stem Cell ◽  
Myelodysplastic Syndrome ◽  
Older Patients ◽  
Advanced Age ◽  
Cell Transplant ◽  
High Grade ◽  
Bone Marrow Fibrosis ◽  
Marrow Fibrosis

Abstract Background:Myelodysplastic syndrome with coexisting bone marrow fibrosis has a dismal prognosis when treated with conventional chemotherapy suggesting that marrow fibrotic grade may be an important prognostic factor. Allogeneic stem cell transplantation (ASCT) has been utilized in young fit patients with this disorder. However outcomes in older patients are unknown. Design and Methods: We conducted a retrospective study to assess the therapeutic efficacy of ASCT in 11 patients with myelodysplastic syndrome with a known co-existing high grade myelofibrosis (n=11) .Data were classified based on cytogenetic risk, conditioning regimen, donor source and cytoreduction pre-transplant. We evaluated rates of engraftment, graft versus host disease and overall survival. Results : Higher median age of 66 years, high cytogenetic risk class and high grade bone marrow fibrosis in our study population was not associated with inferior survival in a univariate analysis. There were no patients who developed graft failure. Cumulative incidence of engraftment achieved at day +30 based on chimersim was 72.7%. The 3 year overall survival was 100% when pre-transplant cytoreduction was used. There were no cases of disease relapse. Conclusions : Among patients with myelodysplastic syndrome and concomitant bone marrow fibrosis, advanced age, a higher grade of fibrosis or cytogenetic complexity did not adversely affect survival after ASCT.This treatment modality should therefore be considered in patients even with advanced age. Disclosures No relevant conflicts of interest to declare.

Validation of IPSS criteria in more than 1,600 MDS patients

2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 7079-7079 ◽  
Author(s):  
F. Quddus ◽  
A. Ahmed ◽  
S. Naqvi ◽  
K. Hasan ◽  
M. Mumtaz ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Survival Time ◽  
Median Survival ◽  
Scoring System ◽  
Median Survival Time ◽  
Bone Marrow Failure ◽  
Prognostic Indicators ◽  
International Prognostic Scoring System ◽  
P Value ◽  
Survival Difference

7079 Myelodysplastic syndromes (MDS) are a diverse group of clonal stem cell disorders characterized by bone marrow failure, dysmyelopoiesis and peripheral cytopenias and affecting predominantly an elderly population. The International Prognostic Scoring System (IPSS) incorporates the number of peripheral cytopenias, percentage of bone marrow blasts and chromosomal abnormalities and assigns a score to predict survival and risk of disease progression to AML. Using the extensive MDS database at the University of Massachusetts we analyzed survival time in relation to IPSS scoring and also its various individual components, i.e. blast percentage, number of cell lines involved and the number of karyotype abnormalities in 1,200+ patients. The overall median survival time in 1,424 MDS patients as a group was 2.9 years. IPSS low group had the longest median survival time of 7.5 years with IPSS Int-1 3.6 years. There was minimal difference in the median survival time between IPSS Int-2 and IPSS high risk group 1.2 and 1.1 years respectively. These results were significant for a P value of <0.0001. The median survival time for blasts <5% was 5.3 years and blast 5–10% was 1.7 years. Interestingly, there was minimal survival difference between median survival time for blasts 11–20% and blasts >20% showing 1.2 years and 1.3 years respectively. Again, these results were significant for a P value of <0.0001. The median survival time for the number of cytopenias involved was also calculated with 0, 1, 2 and 3 numbers of cytopenias showing 6.4 years, 4.4 years, 2.6 years and 1.8 years respectively, with P value of <0.0001. The median survival time for normal karyotype versus one or two karyotype abnormality was 4.9 years, 2.6 years and 2.4 years respectively. Three or more karyotype abnormalities showed a median survival time of 0.8 years. The P value was again significant (<0.0001). Our results not only validate the prognostic value of IPSS scoring system as a whole but also its various individual prognostic indicators. No significant financial relationships to disclose.

Impact of marrow blasts percentage on high-grade myelodysplastic syndrome assessed using revised international prognostic scoring system

2020 ◽  
Vol 99 (3) ◽  
pp. 513-518
Author(s):  
Omar Alkharabsheh ◽  
Mrinal M. Patnaik ◽  
Naseema Gangat ◽  
Kebede H. Begna ◽  
Hassan B. Alkhateeb ◽  
...  
Keyword(s):  
Myelodysplastic Syndrome ◽  
Scoring System ◽  
International Prognostic Scoring System ◽  
High Grade

scholarly journals Eritropoietina Sérica como Marcador Prognóstico em Síndrome Mielodisplásica

2015 ◽  
Vol 28 (6) ◽  
pp. 720 ◽  
Author(s):  
Emília Cortesão ◽  
Rita Tenreiro ◽  
Sofia Ramos ◽  
Marta Pereira ◽  
Paula César ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Overall Survival ◽  
Myelodysplastic Syndromes ◽  
Acute Leukaemia ◽  
Scoring System ◽  
De Novo ◽  
International Prognostic Scoring System ◽  
Lower Survival ◽  
Prognostic Indices ◽  
Serum Erythropoietin

<strong>Introduction:</strong> This myelodysplastic syndromes are a heterogeneous entity characterized by dysplasia, hypercellular bone marrow, cytopenias and risk of transformation to acute leukaemia. Prognostic factors, such as bone marrow fibrosis, lactate dehydrogenase and β2-microglobulin elevation have been described, but treatment is mainly based in the International Prognostic Scoring System.<br /><strong>Material and Methods:</strong> Our aim was to analyze serum´s erythropoietin at diagnosis in de novo myelodysplastic syndromes patients, through its impact in overall survival and possible implementation as prognostic marker. Clinical and laboratorial data from 102 patients with de novo myelodysplastic syndromes diagnosed between October/2009 and March/2014 were collected. Survival analysis was performed according to serum erythropoietin level stratification, using Kaplan-Meier methodology.<br /><strong>Results:</strong> Our 102 patients had a median age of 74 years, with a male:female ratio of 0.8. Mean erythropoietin was significantly lower in refractory cytopenia with unilineage dysplasia patients in contrast with the higher values observed in 5q- syndrome (p &lt; 0.05). Eleven patients progressed to acute leukaemia; these have higher mean erythropoietin values (p &lt; 0.05). In addition, elevated serum erythropoietin was associated with lower survival rates (p = 0.0336). Predictive value of serum erythropoietin was maintained after Cox regression adjustment. In multivariate analysis, serum erythropoietin is an independent survival predictor (p &lt; 0.001).<br /><strong>Discussion:</strong> Serum erythropoietin is a predictive factor for response to therapy with subcutaneous erythropoietin, and patients with myelodysplastic syndromes with higher values of erythropoietin have poorer response to administration of erythropoietin even at higher doses. Our sample shows that serum erythropoietin also has prognostic value, and in all myelodysplastic syndromes subtypes. Moreover, alone or in combination with other factors or prognostic indices, erythropoietin may enhance the prognostic indices such as the International Prognostic Scoring System, since high levels are associated with progression to acute leukemia and hence lower survival.<br /><strong>Conclusion:</strong> This study suggests that increased erythropoietin levels at diagnosis can by itself be a poor prognosis factor in<br />myelodysplastic syndromes patients, with higher values in patients with progression to acute leukaemia and decreased overall survival.

scholarly journals Coalesced Multicentric Analysis of 2,351 Patients With Myelodysplastic Syndromes Indicates an Underestimation of Poor-Risk Cytogenetics of Myelodysplastic Syndromes in the International Prognostic Scoring System

2011 ◽  
Vol 29 (15) ◽  
pp. 1963-1970 ◽  
Author(s):  
Julie Schanz ◽  
Christian Steidl ◽  
Christa Fonatsch ◽  
Michael Pfeilstöcker ◽  
Thomas Nösslinger ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Overall Survival ◽  
Myelodysplastic Syndromes ◽  
Scoring System ◽  
International Prognostic Scoring System ◽  
Prognostic Impact ◽  
Bone Marrow Blast ◽  
Poor Risk ◽  
Blast Count ◽  
The Impact

Purpose The International Prognostic Scoring System (IPSS) remains the most commonly used system for risk classification in myelodysplastic syndromes (MDSs). The IPSS gives more weight to blast count than to cytogenetics. However, previous publications suggested that cytogenetics are underweighted in the IPSS. Here we investigate the prognostic impact of cytogenetic subgroups compared with that of bone marrow blast count in a large, multicentric, international patient cohort. Patients and Methods In total, 2,351 patients with MDS who have records in the German-Austrian and the MD Anderson Cancer Center databases were included and analyzed in univariate and multivariate models regarding overall survival and risk of transformation to acute myeloid leukemia (AML). The data were analyzed separately for patients treated with supportive care without specific therapy, with AML-like chemotherapy, or with other therapy regimens (low-dose chemotherapy, demethylating agents, immune modulating agents, valproic acid, and cyclosporine). Results The prognostic impact of poor-risk cytogenetic findings (as defined by the IPSS classification) on overall survival was as unfavorable as an increased (> 20%) blast count. The hazard ratio (compared with an abnormal karyotype or a bone marrow blast count < 5%) was 3.3 for poor-risk cytogenetics, 4.8 for complex abnormalities harboring chromosomes 5 and/or 7, and 3.1 for a blast count of 21% to 30% (P < .01 for all categories). The predictive power of the IPSS cytogenetic subgroups was unaffected by type of therapy given. Conclusion The independent prognostic impact of poor-risk cytogenetics on overall survival is equivalent to the impact of high blast counts. This finding should be considered in the upcoming revision of the IPSS.

scholarly journals Should We Look beyond Revised International Prognostic Scoring System: A Retrospective Observational Study of Progression of Myelodysplastic Syndrome to Acute Leukemia

2021 ◽  
Vol 42 (05) ◽  
pp. 431-438
Author(s):  
Bangalore Rammohan Nagarjun ◽  
Rajashekar Kalaharaghini ◽  
Jyoti Sawhney ◽  
Pina J. Trivedi ◽  
Karthik Dhandapani ◽  
...  
Keyword(s):  
Acute Leukemia ◽  
Myelodysplastic Syndrome ◽  
Scoring System ◽  
De Novo ◽  
International Prognostic Scoring System ◽  
Cancer Center ◽  
Western India ◽  
Cytogenetic Finding ◽  
Risk Of Progression ◽  
Cytogenetic Profile

Abstract Introduction Myelodysplastic syndrome (MDS) is a clonal stem cell disorder and heterogeneous condition resulting in peripheral cytopenias with marrow dysplasia due to ineffective hematopoiesis. The revised International Prognostic Scoring System (IPSS-R) predicts the risk of progression to acute leukemia (AL). Indian data on MDS and its progression to AL are limited. Additionally, the cytogenetic findings are dictated by patients' racial background. Study intended to analyze the cytogenetic profile of the patients with MDS. Objectives This study aimed to (1) evaluate the clinicohematologic and morphologic spectrum of newly diagnosed MDS cases, (2) evaluate the cytogenetic profile of these cases, and (3) study the cases progressed to AL. Materials and Methods MDS cases diagnosed and followed-up during a 5-year study period, from January 2015 to December 2019, were included in the study and the study was conducted at regional cancer center in Western India. De novo diagnosed MDS cases with complete workup were considered and MDS due to secondary causes were excluded. Baseline clinical, hematologic findings were tabulated along with cytogenetics and risk stratified as per IPSS-R, and their progression was studied. Results A total of 63 cases of de novo MDS were diagnosed over a period of 5 years with 45 cases on follow-up and 15 cases (33.3%) progressed to AL. Maximum number of cases belonged to MDS-excess blast (EB) category accounting to 48 cases (76.1%). Apparently normal karyotyping was the commonest cytogenetic finding in 33 MDS cases (61.2%) and in 8 cases that progressed to AL (53.4%). Conclusion MDS cases diagnosed at relatively early age were at higher risk of progression to AL. Majority of the cases that progressed to AL were risk stratified in high and very high risk groups and 10 cases which progressed to AL belonged to good category, interestingly apparent normal karyotyping was the commonest cytogenetic finding in more than 50% of the cases progressed to AL. Molecular mutations could only explain this progression and studies integrating molecular mutations with present IPSS-R scoring system should be conducted, as it could translate into better risk stratification and help in early identification and better management of cases at risk in progression to AL.

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