Validation of WHO classification-based Prognostic Scoring System (WPSS) for myelodysplastic syndromes and comparison with the revised International Prognostic Scoring System (IPSS-R). A study of the International Working Group for Prognosis in Myelodysplasia (IWG-PM)

Leukemia ◽  
2015 ◽  
Vol 29 (7) ◽  
pp. 1502-1513 ◽  
Author(s):  
M G Della Porta ◽  
H Tuechler ◽  
L Malcovati ◽  
J Schanz ◽  
G Sanz ◽  
...  
Keyword(s):  
Myelodysplastic Syndromes ◽  
Working Group ◽  
Scoring System ◽  
Who Classification ◽  
International Prognostic Scoring System ◽  
International Working Group

Marrow Fibrosis in Myelodysplastic Syndromes - Its Significance in the Context of the WHO Classification of Disease and the International Prognostic Scoring System.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 1446-1446
Author(s):  
Guntram Buesche ◽  
Arnold Ganser ◽  
Ludwig Wilkens ◽  
Brigitte Schlegelberger ◽  
Hartmut Hecker ◽  
...  
Keyword(s):  
Survival Time ◽  
Myelodysplastic Syndromes ◽  
Scoring System ◽  
Who Classification ◽  
World Health ◽  
International Prognostic Scoring System ◽  
Bone Marrow Biopsies ◽  
Health Organization ◽  
Marrow Fibrosis

Abstract Marrow fibrosis (MF) is rarely considered in myelodysplastic syndromes (MDS) although the frequency of this complication ranges from 10 to 50 % in the few reports on this issue, and there are no data on occurrence and significance of this complication in the context of the International Prognostic Scoring System (IPSS) and the World Health Organization (WHO) classification of disease. In a retrospective study, diagnostic bone marrow biopsies from a total of 936 patients with MDS were examined for MF and its relevance to the course of disease. Frequency of MF varied markedly between different types of MDS ranging from 3 % (RARS) to 37 % (MDS, therapy-related; WHO classification, P < 0.000005). Risk of MF furthermore correlated with multilineage dysplasia (P < 0.000005). However, there was no obvious correlation to the IPSS or to karyotype abnormalities. The survival time of patients was significantly reduced by about 50 % from 11 (RAEB-1/-2) - 55 (RARS, RCMD-RS) down to 6 (RAEB-1/-2) - 33 months (RARS, RCMD-RS) in median when MF was detected independently of the IPSS and the classification of disease (FAB, WHO; P = 0.0001). We conclude that MF is an unfavorable complication of MDS significantly shortening the survival time of patients independently of the IPSS and the classification of disease.

scholarly journals New Comprehensive Cytogenetic Scoring System for Primary Myelodysplastic Syndromes (MDS) and Oligoblastic Acute Myeloid Leukemia After MDS Derived From an International Database Merge

2012 ◽  
Vol 30 (8) ◽  
pp. 820-829 ◽  
Author(s):  
Julie Schanz ◽  
Heinz Tüchler ◽  
Francesc Solé ◽  
Mar Mallo ◽  
Elisa Luño ◽  
...  
Keyword(s):  
Acute Myeloid Leukemia ◽  
Myelodysplastic Syndromes ◽  
Working Group ◽  
Scoring System ◽  
Myeloid Leukemia ◽  
External Validation ◽  
International Prognostic Scoring System ◽  
Prognostic Impact ◽  
Bootstrap Analysis ◽  
Acute Myeloid

Purpose The karyotype is a strong independent prognostic factor in myelodysplastic syndromes (MDS). Since the implementation of the International Prognostic Scoring System (IPSS) in 1997, knowledge concerning the prognostic impact of abnormalities has increased substantially. The present study proposes a new and comprehensive cytogenetic scoring system based on an international data collection of 2,902 patients. Patients and Methods Patients were included from the German-Austrian MDS Study Group (n = 1,193), the International MDS Risk Analysis Workshop (n = 816), the Spanish Hematological Cytogenetics Working Group (n = 849), and the International Working Group on MDS Cytogenetics (n = 44) databases. Patients with primary MDS and oligoblastic acute myeloid leukemia (AML) after MDS treated with supportive care only were evaluated for overall survival (OS) and AML evolution. Internal validation by bootstrap analysis and external validation in an independent patient cohort were performed to confirm the results. Results In total, 19 cytogenetic categories were defined, providing clear prognostic classification in 91% of all patients. The abnormalities were classified into five prognostic subgroups (P < .001): very good (median OS, 61 months; hazard ratio [HR], 0.5; n = 81); good (49 months; HR, 1.0 [reference category]; n = 1,809); intermediate (26 months; HR, 1.6; n = 529); poor (16 months; HR, 2.6; n = 148); and very poor (6 months; HR, 4.2; n = 187). The internal and external validations confirmed the results of the score. Conclusion In conclusion, these data should contribute to the ongoing efforts to update the IPSS by refining the cytogenetic risk categories.

Arsenic Trioxide in Patients With Myelodysplastic Syndromes: A Phase II Multicenter Study

2006 ◽  
Vol 24 (16) ◽  
pp. 2465-2471 ◽  
Author(s):  
Norbert Vey ◽  
Andre Bosly ◽  
Agnes Guerci ◽  
Walter Feremans ◽  
Herve Dombret ◽  
...  
Keyword(s):  
Arsenic Trioxide ◽  
Myelodysplastic Syndromes ◽  
Scoring System ◽  
Lower Risk ◽  
International Prognostic Scoring System ◽  
Risk Category ◽  
Moderate Activity ◽  
Loading Dose ◽  
Median Response ◽  
System Risk

Purpose Evaluation of the safety and efficacy of arsenic trioxide in patients with myelodysplastic syndromes (MDS). Patients and Methods MDS patients diagnosed according to standard French-American-British criteria received a loading dose of 0.3 mg/kg per day of arsenic trioxide for 5 days followed by a maintenance dose of 0.25 mg/kg arsenic trioxide twice weekly for 15 weeks. Patients were divided into two cohorts: lower-risk MDS (International Prognostic Scoring System risk category low or intermediate 1) and higher-risk MDS (International Prognostic Scoring System risk category intermediate 2 or high). Modified International Working Group criteria were used for response evaluation. Results Of 115 patients enrolled and treated in the study, 67% of patients were transfusion dependent at baseline; median age was 68 years. Most treatment-related adverse events were mild to moderate. The overall rate of hematologic improvement (intent-to-treat) was 24 (19%) of 115, including one complete and one partial response in the higher-risk cohort. The hematologic response rates were 13 (26%) of 50 and 11 (17%) of 64 in patients with lower-risk and higher-risk MDS, respectively. Major responses were observed in all three hematologic lineages; 16% of RBC transfusion-dependent patients and 29% of platelet transfusion-dependent patients became transfusion independent. At data cut off, the median response duration was 3.4 months, with responses ongoing in nine patients. Conclusion Arsenic trioxide treatment consisting of an initial loading dose followed by maintenance therapy has moderate activity in MDS, inducing hematologic responses in both lower- and higher-risk patients. This activity combined with a manageable adverse effect profile warrants the additional study of arsenic trioxide, particularly in combination therapy, for the treatment of patients with MDS.

scholarly journals Application of the revised International Prognostic Scoring System for myelodysplastic syndromes in Argentinean patients

2013 ◽  
Vol 93 (4) ◽  
pp. 705-707 ◽  
Author(s):  
Carolina B. Belli ◽  
Yesica Bestach ◽  
Mario Giunta ◽  
Marcelo Iastrebner ◽  
Isabel Santos ◽  
...  
Keyword(s):  
Myelodysplastic Syndromes ◽  
Scoring System ◽  
International Prognostic Scoring System

Evaluation of the Intra-LABORATORY Reproducibility of Percentage of Blast Counting In MYELODYSPLASIA

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4901-4901
Author(s):  
Soraya Wuilleme ◽  
Marion Eveillard ◽  
Sophie Barillet ◽  
Françoise Accard ◽  
Hervé Avet-Loiseau ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Scoring System ◽  
Who Classification ◽  
Conflicts Of Interest ◽  
Scoring Systems ◽  
Poor Quality ◽  
International Prognostic Scoring System ◽  
Inter Observer Variability ◽  
Morphological Criteria ◽  
Blast Cells

Abstract Abstract 4901 In the two International Prognostic Scoring Systems of Myelodysplastic Syndrom (MDS), the percentage of BLAST cells in the Bone Marrow is the most important parameter implicated in score: either directly in the IPSS (International Prognostic Scoring System) (1) or indirectly in the WPSS (Who classification-based prognostic scoring system) (2). In these two systems, as in the recommendations of the WHO 2001 (3), the morphological criteria defining blasts cells compared to promyelocyte cells are not specified. In 2005, the IWGM-MDS (International Working Group on Morphology of myelodysplastic syndrome) (4-6) has established morphological criteria defining Blasts cells. Our objective in this study is to evaluate the reproducibility, among five observers of our laboratory, of the counting of the marrow blasts of 73 myelodysplasia. This study was conducted in several stages. 1st step was to test the implementation of the MDS-Foundation (www.mds-foundation.org/virtualmicroscopy) by 5 observers. The 2nd step was to perform correlation test of the selected cells from RAEB (selected and stained in our laboratory conditions), for the 5 observers. Finally Step 3 was to assess the correlation of blast percentage of 73 bone marrow smears from MDS patients, selected due to the presence of an excess of blast cells in the first reading on the bone aspiration. Our results show that the correlation on counting blasts is generally satisfactory (percentage agreement: Test 1 = 86% and test 2 = 94%), while the concordance on the counting blasts of bone marow smears of 73 MDS patients and concordance on the WHO classification seems less satisfactory (agreement 3/5 observers. = 95% but agreed to 4–5/5 observers = 64%). These results can be explained partly by the inter-observer variability and by the variability of some parameters specific to smear marrow (poor quality smears, the staining and/or poverty of smears). In conclusion, the evaluation of the blasts in the MDS must be achieved: (i) at least 500 cells counted (ii) by at least two different observers (iii) by a third observer in discordant case. Despite these recommendations, the assessment of the blasts in myelodysplasia is difficult to achieve in many cases. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Early lenalidomide treatment for low and intermediate-1 International Prognostic Scoring System risk myelodysplastic syndromes with del(5q) before transfusion dependence

2015 ◽  
Vol 4 (12) ◽  
pp. 1789-1797 ◽  
Author(s):  
Esther N. Oliva ◽  
Michael Lauseker ◽  
Maria Antonietta Aloe Spiriti ◽  
Antonella Poloni ◽  
Agostino Cortelezzi ◽  
...  
Keyword(s):  
Myelodysplastic Syndromes ◽  
Scoring System ◽  
International Prognostic Scoring System ◽  
System Risk
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