High Predictive Value of the Revised International Prognostic Scoring System (IPSS-R): An External Analysis of 646 Patients From a Multiregional Italian MDS Registry

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 1702-1702 ◽  
Author(s):  
Emanuela Messa ◽  
Daniela Gioia ◽  
Andrea Evangelista ◽  
Bernardino Allione ◽  
Emanuele Angelucci ◽  
...  

Abstract Abstract 1702 Background: Prognostic assessment has a crucial role in clinical evaluation of patients (pts) affected by myelodysplastic syndrome (MDS). Recently a Revised International Prognostic Scoring System (IPSS-R) has been developed (Greenberg et al, 2012) to improve the standard IPSS (Greenberg et al, 1997): it identifies five different prognostic categories mainly based on stratification of cytogenetic risk. Another prognostic score proposed in clinical practice is WPSS, based on transfusion dependency and WHO morphologic classification (Malcovati et al, 2005) subsequently modified (rWPSS) introducing level of hemoglobin in lieu of the previous not well defined variable of transfusion dependency (Malcovati et al, 2011). Aims: Aim of our study was to evaluate in a cohort of MDS pts enrolled in the Multiregional Italian MDS Registry the prognostic value of IPSS-R respect to IPSS and compare it with both WPSS and rWPSS. Materials and methods: Among the 1918 MDS pts enrolled in the Multiregional Italian MDS Registry from 1999 to 2012 we excluded all the cases already included in the IWG-PM database that generated the IPSS-R. We thus obtained a cohort of 646 pts with complete follow up. We evaluated the prognostic power of IPSS-R respect to IPSS, WPSS and rWPSS respectively by Harrell's C statistics, analyzing as endpoints overall survival (OS), leukemic evolution (LE) and progression free survival (PFS). For LE we considered leukemic evolution as an event, while all the other causes of death were competing events. For PFS we consider either leukemic evolution or death for any causes as an event. Results: Median age of MDS patients was 75 years (interquartile range: 69–80 years). 378 (59%) out of 646 pts were males. WHO classification was as follows: 33% RCMD, 10% RAEB-1, 9% RAEB-2, 6% CMML, 2% MDS-U, the remaining 40% were RARS, RA, isolated 5q deletion. Median follow up of censored pts was 17 months. According to IPSS score, 47% of pts were low risk, 39% Int-1, 10% Int-2 and 4% high risk. WPSS stratification was as follows: 31% were very low (VL) risk, 37% low (L), 19% intermediate (I), 11% high (H) and 2% very high (VH). By applying rWPSS stratification we obtained 30% VL, 35% L, 17% I, 15% H and 3% VH risk pts. IPSS-R risk stratification was as follows: 20% VL, 46% L, 20% I, 9% H and 5% VH risk pts. OS was analyzed according to the different scores by Kaplan-Meyer method. All prognostic systems allowed the identification of survival curves with significant differences among the different categories of risk stratification. IPSS-R application defined OS curves which better defined patients prognostic categories as shown in fig 1. In fact Harrel's C statistics demonstrated a better predictive value of the IPSS-R respect to IPSS, but also respect to WPSS and rWPSS (C=0,73; 0,63; 0,65; 0,64 respectively). Similar results have been obtained also considering time to LE (fig 2). Harrel's C statistics for LE was 0,84; 0,76; 0,78; 0,77 respectively in IPSS-R, IPSS, WPSS, rWPSS risk stratification groups. Moreover, we analyzed PFS outcomes (fig 3). Also in this case, IPSS-R showed the greatest prognostic power: Harrel's C statistics was 0,76; 0,67; 0,66; 0,69 respectively in IPSS-R, IPSS, WPSS, rWPSS risk stratification groups. Conclusions: In our hands, IPSS-R score demonstrated a better prognostic power respect to previously published prognostic systems (IPSS, WPSS, rWPSS). The cohort of MDS patients we employed to validate the new prognostic scoring system has a short follow up (17 months), due to the exclusion of cases already used to establish the IPSS-R system, and the majority of these are lower risk ones. We can conclude that a careful classification based on cytogenetic examination improve the prognostic power of the score. Thus, IPSS-R is confirmed to be a refined tool, easily applicable in real life and empowered respect to the currently used scores to define MDS patient prognosis. Disclosures: Saglio: Bristol-Myers Squibb: Consultancy, Speakers Bureau.

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 5217-5217
Author(s):  
Jae Hyeon Park ◽  
Si Nae Park ◽  
Jiseok Kwon ◽  
Kyongok Im ◽  
Jung Ah Kim ◽  
...  

Abstract Introduction The International Prognostic Scoring System (IPSS) was recently revised (IPSS-R). IPSS-R was developed using a large cohort of patients received supportive therapy, so we validated the new prognostic score system in Korean patients. IPSS-R emphasizes initial cytogenetic abnormalities, but the risk of clonal evolution is not well identified in follow up of MDS patients. Methods Data of 88 MDS patients were collected retrospectively and verified by chart review. And we also collected cytogenetic analysis results performed with bone marrow study. Results By IPSS-R cytogenetic scoring system, 2 (2.3%), 41 (46.6%), 22 (25.0%), 5 (5.7%), and 18 (20.5%) patients were classified as very good, good, intermediate, poor, and very poor, respectively. According IPSS, 5 (5.7%), 50 (56.8%), 19 (21.6%), and 14 (15.9%) patients were classified as low, intermediate-1, intermediate-2, and high, respectively. According to IPSS-R, 2 (2.3%), 13 (14.8%), 28 (31.8%), 25(28.4%), and 20 (22.7%) patients were reclassified as very low, low, intermediate, high, and very high, respectively. Median 3 years overall survival of patients intermediate, high and very high IPSS-R are 36 (95%CI 34-39), 32 (95%CI 8 – 56), and 13 (95%CI 6-21), respectively (P = 0.004). Clonal evolutions were occurred in 22 patients and they were classified by IPSS-R cytogenetic scoring system: 11 (50.0%) good, 8 (36.4%) intermediate, 1 (4.5%) poor, 2 (9.1%) very poor. Prior clonal evolution was considered as except concurrent occurrence of clonal evolution and acute myeloid leukemia (AML) transformation. These 17 patients have significant correlation with AML transformation (P = 0.010) and mortality (P = 0.021). Conclusion IPSS-R gives more refined prognostic discrimination and can be applicable in Korean MDS patients. In addition, the clonal evolution occurring during follow up should be considered as a risk factor of AML transformation and mortality. Disclosures: No relevant conflicts of interest to declare.


Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 1730-1730
Author(s):  
Jose F Falantes ◽  
Cristina Calderón ◽  
Ildefonso Espigado ◽  
Dora Alonso ◽  
Antonio Martín Noya ◽  
...  

Abstract Abstract 1730 Background: Prognosis of patients (pts) with myelodysplastic syndrome (MDS) is very heterogeneous. Currently, several classifications for MDS as well as different scoring systems are available, that allow to stratify pts in terms of overall survival and risk of transformation to acute myeloid leukemia (AML). However, the subgroup of pts categorized as lower-risk MDS (International Prognostic Scoring System; IPSS, low or intermediate-1 or less than 10% bone marrow blasts) are not properly stratified by IPSS. Factors as age, severity of cytopenias and transfusion dependency (TD) are not considered in classical scoring systems except for TD in World Health Organization (WHO)-Based Prognosis Scoring System (WPSS). Recently, García-Manero et al reported a prognostic score for lower-risk MDS, identifying age (>60y), degree of cytopenias, bone marrow blasts >4% and karyotype as variables which significantly influenced on survival. Three groups of pts were identified with significant differences in estimated survival. Methods: We analyzed baseline characteristics of 335 lower-risk MDS pts from a single institution and their impact on survival and risk for AML progression in a two decade time period (1990–2010). Median age was 72 years (range: 18–93). Most frequent MDS subtypes were refractory anemia (RA) and RA with ringed sideroblasts. Treatment approach included best supportive care and/or observation in 85% pts. Patients on lenalidomide, 5 azacitidine or allogeneic stem cell recipients were excluded from analysis. Baseline characteristics are shown in table 1Results: After a mean follow-up of 39 months, severity of cytopenias: anemia <10 g/dL [HR=1.701 (95% CI: 1.173–2.467), p=0.005], and platelets <50×10e /L [HR=1.853 (95% CI: 1.287–2.667), p=0.001], age >60y [HR=1.909 (95% CI: 1.256–2.902), p=0.002], bone marrow blast percentage (<5% vs 5–9%) [HR=1.288 (95% CI: 1.2–3.045), p=0.006] and TD [HR=1.548 (95% CI: 1.092–2.195), p=0.014], significantly influenced on outcome in multivariate analysis. The combination of these variables allowed to develop a new score system which categorizes classic lower-risk MDS pts in three different groups with median survival of 84 months (95% CI 40–127) for group 1, 41 months for group 2 (95% CI 28–53) and 13 months for group 3 (95% CI 10–15). Estimated 4-year survival was 65%, 38% and 10% for pts in groups 1, 2 and 3 respectively (p<0.001, Figure 1). At last follow-up, 47 pts (14%) progressed to AML. TD and BM blasts 5–9% were the only factors associated with increased risk of AML progression. In addition, this score also stratified pts depending on their risk for AML, estimated at 2 years in 6%, 13% and 31% for groups 1, 2 and 3 respectively (p=0.001, Figure 2). Conclusion: We describe a simplified score, based on data easily available in most institutions, which allows to discriminate different subgroups with different outcome within the lower-risk MDS, identifying in this subset, pts with a poorer prognosis in which an early and more aggressive intervention could possibly be required to improve survival or disease progression. Abbreviations: RA, Refractory anemia; RARS, Refractory anemia with ringed sideroblasts; RCMD, Refractory cytopenia with multilineage dysplasia; RAEB-1, Refractory anemia with excess blast; CMML, Chronic myelomonocytic leukemia; MDS-U, Myelodysplastic syndrome unclassificable; ANC, Absolute neutrophil count; IPSS, International Prognostic Scoring System; NA, Not assessable; TD,Transfusion dependency at diagnosis. Disclosures: No relevant conflicts of interest to declare.


2003 ◽  
Vol 90 (08) ◽  
pp. 344-350 ◽  
Author(s):  
Alexander Niessner ◽  
Senta Graf ◽  
Mariam Nikfardjam ◽  
Walter Speidl ◽  
Renate Huber-Beckmann ◽  
...  

SummaryThrombus formation after rupture of an atherosclerotic plaque plays a crucial role in coronary artery disease (CAD). A decreased endogenous fibrinolytic system and prothrombotic factors are supposed to influence coronary thrombosis. It was our aim to investigate the predictive value of tissue plasmino-gen activator (t-PA) antigen, von Willebrand Factor, Lipoprotein (a) and anti-cardiolipin antibodies for major adverse coronary events in patients with stable CAD in a prospective cohort study of more than 10 years.We observed 141 patients with angiographically proven CAD for a median follow-up period of 13 years. t-PA antigen was the only marker predicting coronary events (logistic regression, p = 0.044) with a poor prognosis for patients in the 5th quintile with an odds ratio of 7.3 (compared to the 1st quintile). The odds ratio even increased to 10.0 for coronary events associated with the “natural course” of CAD excluding events due to restenosis. t-PA antigen had a slightly higher prognostic power (ROC curve; AUC = 0.69) than fasting glucose (AUC = 0.68) and cholesterol (AUC = 0.67). Triglycerides influenced plasma levels of t-PA antigen (regression, p < 0.001). The predictive value of t-PA antigen remained significant after adjustment for inflammation (logistic regression, p = 0.013) and extent of CAD (p = 0.045) but disappeared adjusting for insulin resistance (p = 0.12).In conclusion t-PA antigen predicted coronary events during a very long-term follow-up with a comparable prognostic power to established cardiovascular risk factors. Markers of insulin resistance influenced t-PA antigen and its predictive value.Part of this paper was originally presented at the joint meetings of the 16th International Congress of the International Society of Fibrinolysis and Proteolysis (ISFP) and the 17th International Fibrinogen Workshop of the International Fibrinogen Research Society (IFRS) held in Munich, Germany, September 2002.


2014 ◽  
Vol 170 (6) ◽  
pp. 837-846 ◽  
Author(s):  
Charlotte Lepoutre-Lussey ◽  
Dina Maddah ◽  
Jean-Louis Golmard ◽  
Gilles Russ ◽  
Frédérique Tissier ◽  
...  

ObjectiveCervical ultrasound (US) scan is a key tool for detecting metastatic lymph nodes (N1) in patients with papillary thyroid cancer (PTC). N1-PTC patients are stratified as intermediate-risk and high-risk (HR) patients, according to the American Thyroid Association (ATA) and European Thyroid Association (ETA) respectively. The aim of this study was to assess the value of post-operative cervical US (POCUS) in local persistent disease (PD) diagnosis and in the reassessment of risk stratification in N1-PTC patients.DesignRetrospective cohort study.MethodsBetween 1997 and 2010, 638 N1-PTC consecutive patients underwent a systematic POCUS. Sensitivity, specificity, negative predictive value (NPV), and positive predictive value (PPV) of POCUS for the detection of PD were evaluated and a risk reassessment using cumulative incidence functions was carried out.ResultsAfter a median follow-up of 41.6 months, local recurrence occurred in 138 patients (21.6%), of which 121 were considered to have PD. Sensitivity, specificity, NPV, and PPV of POCUS for the detection of the 121 PD were 82.6, 87.4 95.6, and 60.6% respectively. Cumulative incidence of recurrence at 5 years was estimated at 26% in ETA HR patients, 17% in ATA intermediate-risk patients, and 35% in ATA HR patients respectively. This risk fell to 9, 8, and 11% in the above three groups when the POCUS result was normal and to <6% when it was combined with thyroglobulin results at ablation.ConclusionPOCUS is useful for detecting PD in N1-PTC patients and for stratifying individual recurrence risk. Its high NPV could allow clinicians to tailor follow-up recommendations to individual needs.


Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4408-4408
Author(s):  
German R. Stemmelin ◽  
Marta Dragosky ◽  
Adriana Vitriu ◽  
maria Julia Caffaro ◽  
Miguel A Pavlovsky ◽  
...  

Abstract Different treatment guidelines suggest that advanced follicular lymphoma (AFL) subjects should be treated only when meeting criteria treatment, such as GELF, are present. Conversely, when absent the watch and wait (W&W) approach is recommended. However, in our country, we had the impression that in real life, a high percentage of patients without the above-mentioned criteria were treated. With the purpose of unravelling the medical approach of AFL patients at diagnosis and subsequent evolution, the Lymphoma Subcommittee of the Argentinian Society of Haematology undertook this retrospective survey. Results: From years 2006 to 2014 305 patients from 23 institutions were included. GELF criteria were encountered in 62% of patients at diagnosis and all of them were treated with immunochemotherapy (ICT). Among the 116 (38%) patients without meeting GELF criteria (GELF negative group), in only 30 (26%) W&W was the approach chosen, while the rest received ICT. The survey questionnaire revealed that own assessment of the treating physician was the main reason for treating the GELF negative group. In the W&W group, 60% required ICT at a mean of 17 months, being 15% of them transformed to DLBCL at time of treatment. The 89% of cases (271/305) received ICT at some time; 66% R-CHOP, 29% R-CVP, and 5% other regimes. Patient median age receiving R-CHOP and R-CVP was 57 and 62 years (p<.01), respectively. Rituximab maintenance (RM) was added to ICT in 64% of cases. For the whole group, with a median follow up of 36 months, the overall survival (OS) was 95% and progression free survival (PFS) 68%. Comparing GELF negative and positive groups, PFS was better for GELF negative group, 87% vs 61% (p <.01). There was no difference in OS. Within the GELF negative group, OS was not different between patients treated at the time of diagnosis vs those in which a W&W approach was chosen. Conclusion: 1) When comparing with international reports, the percentage (62%) of patients with positive GELF criteria was higher at diagnosis. This fact may be due to delay in access to health care; 2) we found a remarkable discrepancy among guidelines recommendations and real life medical behaviour. Three out of four patients received treatment at diagnosis, when W&W ought to have been the guideline-recommended approach; 3) R-CHOP was the most used ICT scheme, while R-CVP was mostly reserved for the elderly. RM was indicated in the majority of patients, particularly after year 2011; 4) despite acknowledging the methodological limitations of this retrospective analysis, a high tumor mass (GELF positive) picture conferred a worse prognosis in term of PFS, while a W&W approach did not affect the OS for the GELF negative group. Disclosures Riveros: Roche: Speakers Bureau.


2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 1077-1077 ◽  
Author(s):  
Iris Schrader ◽  
Frank Gerhard Foerster ◽  
Andreas Schneeweiss ◽  
Matthias Geberth ◽  
Lars Hahn ◽  
...  

1077 Background: 1st-line BEV combined with weekly PAC significantly improves progression-free survival (PFS) and response rate (RR) vs PAC alone in HER2-negative mBC, as shown in E2100. We analyzed data from a German routine oncology practice study of 1st-line BEV–PAC according to prognostic factors. Methods: Pts who had received no prior chemotherapy for mBC received BEV–PAC according to the European label. Efficacy and safety were documented for up to 1 y (or until progression, death, or BEV discontinuation if earlier) with additional long-term follow-up. Efficacy was analyzed in clinically important subgroups. Results: Efficacy data were available for 818 pts. The median duration of follow-up was 11.4 mo. The composition of the pt population with respect to the subgroups below was generally similar to the population treated in E2100, except for a higher proportion of pts with visceral disease or metastases in <3 organs. RR was very similar across all subgroups analyzed. Differences in median PFS and OS were generally in line with the differing prognoses according to clinical characteristics. Conclusions: These data suggest that 1st-line BEV–PAC is typically associated with median PFS >9 mo in the real-life setting, irrespective of baseline characteristics. [Table: see text]


2020 ◽  
Vol 38 (6_suppl) ◽  
pp. 738-738
Author(s):  
Jacqueline T Brown ◽  
Yuan Liu ◽  
Dylan J. Martini ◽  
Julie M. Shabto ◽  
Elise Hitron ◽  
...  

738 Background: The current risk models for mRCC were developed for patients treated with targeted therapy. The mGPS incorporates albumin and C-reactive protein and may serve as a composite prognostic biomarker in mRCC in the era of CPI. Methods: We conducted a retrospective analysis of patients with mRCC treated with CPI (anti-PD1 or PD-L1 agents) at Winship Cancer Institute between 2015-2018. Overall survival (OS) and progression-free survival (PFS) were defined as months from CPI initiation to death or clinical/radiographic progression, respectively. mGPS was defined as a summary score with one point given for CRP > 10 mg/L and/or albumin < 3.5 g/dL. Univariate (UVA) and multivariate (MVA) analyses were carried out for OS and PFS using Cox proportional hazard model. Results: A total of 78 eligible patients were included with a median follow up of 30.7 months. Median age was 66 years (range = 38-82), 64% were male, 78% had clear cell histology, and 76% received anti-PD-1 monotherapy. Higher mGPS at baseline was significantly associated with worse OS while at week 6 was associated with worse OS and PFS (Table). Conclusions: A higher mGPS score in the early course of CPI treatment was associated with worse survival in patients with mRCC. These results should be validated in a larger, prospective study.[Table: see text]


2021 ◽  
Vol 10 ◽  
Author(s):  
Ying Fang ◽  
Juan Guo ◽  
Dong Wu ◽  
Ling-Yun Wu ◽  
Lu-Xi Song ◽  
...  

Despite the improvements in prognostication of the revised International Prognostic Scoring System (IPSS-R) in myelodysplastic syndrome (MDS), there remain a portion of patients with lower risk (low/intermediate risk, LR) but poor prognostics. This study aimed to evaluate the relative contribution of mutational status when added to the IPSS-R, for estimating overall survival (OS) and progression-free survival (PFS) in patients with LR-MDS. We retrospectively analyzed clinical and laboratory variables of 328 patients diagnosed with MDS according to the FAB criteria. Twenty-nine-gene NGS assay was applied to bone marrow samples obtained at diagnosis. 233 (71.04%) patients were classified as LR-MDS. Univariate analysis showed association between inferior outcome (OS and PFS) and presence of JAK2 (p = 0.0177, p = 0.0002), RUNX1 (p = 0.0250, p = 0.0387), and U2AF1 (p = 0.0227, p = 0.7995) mutations. Multivariable survival analysis revealed JAK2 (p &lt; 0.0001) and RUNX1 (p = 0.0215) mutations were independently prognostic for PFS in LR-MDS. Interestingly, bone marrow blast &gt;1.5% could further predict disease progression of patients with LR-MDS (HR 8.06, 95%CI 2.95–22.04, p &lt; 0.0001). Incorporation of JAK2, RUNX1 mutation and bone marrow blast in the IPSS-R can improve risk stratification in patients with LR-MDS. In summary, our result provided new risk factors for LR-MDS prognostics to identify candidates for early therapeutic intervention.


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