scholarly journals Revisiting guidelines for integration of flow cytometry results in the WHO classification of myelodysplastic syndromes—proposal from the International/European LeukemiaNet Working Group for Flow Cytometry in MDS

Leukemia ◽  
2014 ◽  
Vol 28 (9) ◽  
pp. 1793-1798 ◽  
Author(s):  
A Porwit ◽  
◽  
A A van de Loosdrecht ◽  
P Bettelheim ◽  
L Eidenschink Brodersen ◽  
...  
Keyword(s):  
Flow Cytometry ◽  
Myelodysplastic Syndromes ◽  
Working Group ◽  
Who Classification

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.

Hematopathological Concepts and Controversies in the Diagnosis and Classification of Myelodysplastic Syndromes

Hematology ◽  
2006 ◽  
Vol 2006 (1) ◽  
pp. 199-204 ◽  
Author(s):  
James W. Vardiman
Keyword(s):  
Bone Marrow ◽  
Myelodysplastic Syndromes ◽  
Who Classification ◽  
Diagnostic Dilemma ◽  
Genetic Studies ◽  
Diagnostic Uncertainty ◽  
Diagnostic Problems ◽  
Diagnosis And Classification

Abstract Although the diagnosis and classification of most cases of the myelodysplastic syndromes (MDS) is usually accomplished without difficulty, a minority of cases may pose diagnostic problems. In many cases the diagnostic dilemma can be solved by adhering to basic guidelines recommended for evaluation of patients suspected of having MDS, and in particular to the quality of the blood and bone marrow specimens submitted for morphologic, immunophenotypic and genetic studies. In other cases, such as patients who have hypocellular MDS or MDS with fibrosis, the criteria for making a diagnosis may be difficult if not impossible to apply, and in still others the diagnostic uncertainty is because the minimal criteria necessary to establish the diagnosis of MDS are not always clearly stated. In this review, some of these diagnostic problems are addressed and some general guidelines for resolving them are suggested. In addition, data are presented that illustrate that the WHO classification offers a valuable tool in the diagnosis and classification of MDS.

Tolerability and hematologic improvement assessed using three alternative dosing schedules of azacitidine in patients with myelodysplastic syndromes

2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 7083-7083 ◽  
Author(s):  
R. M. Lyons ◽  
T. Cosgriff ◽  
S. Modi ◽  
H. McIntyre ◽  
C. L. Beach ◽  
...  
Keyword(s):  
Myelodysplastic Syndromes ◽  
Working Group ◽  
Open Label ◽  
Dosing Schedule ◽  
Fab Classification ◽  
Open Label Trial ◽  
Grade 3 ◽  
Treatment Related Mortality ◽  
Related Mortality

7083 Background: At a dosing schedule of 75 mg/m2/day SC for 7 days every 4 weeks, azacitidine is an effective and safe treatment (Tx) for patients (pts) with myelodysplastic syndromes (MDS) (JCO 2002; 20:2429). An alternative dosing schedule that eliminates the need for weekend dosing would be more convenient to pts and clinicians. Methods: In this phase II, multicenter, open-label trial, pts with MDS were randomized to 1 of 3 regimens that were repeated every 4 weeks: AZA 5–2-2 (75 mg/m2/day × 5 days, followed by 2 days no Tx, followed by 75 mg/m2/day × 2 days), AZA 5–2-5 (50 mg/m2/day × 5 days, followed by 2 days no Tx, followed by 50 mg/m2/day × 5 days) or AZA 5 (75 mg/m2/day × 5 days). To determine if response/improvement according to International Working Group criteria (Blood 2000; 96:3671) can be maintained after 6 cycles, the study was amended to include a 12-month maintenance comparing AZA 5 every 4 weeks with AZA 5 every 6 weeks. Results: As of Nov. 30, 2006, 138 pts have been randomized to AZA 5–2-2 (n=46), AZA 5–2-5 (n=47) and AZA 5 (n=45). Most pts are RA (43%) or RAEB (30%), based on FAB classification. Of 104 pts who have received =2 cycles of Tx, hematologic improvement (major or minor in at least 1 cell line) occurred in 63% (65) of the patients ( Table ). Of these pts, 14% had a bi-lineage (AZA 5–2-2: 11%, AZA 5–2-5: 10%, AZA 5: 22%) and 6% had a tri-lineage AZA 5–2-2: 6%, AZA 5–2-5: 7%, AZA 5: 5%) response (based on any improvement). Ongoing pts in the study include AZA 5–2-2: 41% (19/46), AZA 5–2-5: 47% (22/47), and AZA 5: 58% (26/45). No treatment-related mortality has been reported. Most Tx-related grade 3 or 4 events were hematological (AZA 5–2-2: 39%, AZA 5–2-5: 24%, AZA 5: 16%). Updated data, including several pts who have completed at least 6 cycles maintenance, will be available at the time of the meeting. Conclusions: These data indicate that the 3 alternative azacitidine dosing schedules are safe, effective, and similar in efficacy with the FDA-approved regimen. [Table: see text] [Table: see text]

P-051 Flow cytometry in myelodysplastic syndromes: A report from an international consortium and the European LeukemiaNet Working Group

2013 ◽  
Vol 37 ◽  
pp. S45
Author(s):  
T.M. Westers ◽  
W. Kern ◽  
R. Ireland ◽  
M.C. Béné ◽  
M.G. Della Porta ◽  
...  
Keyword(s):  
Flow Cytometry ◽  
Myelodysplastic Syndromes ◽  
Working Group ◽  
International Consortium

scholarly journals Standardization of flow cytometry in myelodysplastic syndromes: a report from an international consortium and the European LeukemiaNet Working Group

Leukemia ◽  
2012 ◽  
Vol 26 (7) ◽  
pp. 1730-1741 ◽  
Author(s):  
T M Westers ◽  
R Ireland ◽  
W Kern ◽  
C Alhan ◽  
J S Balleisen ◽  
...  
Keyword(s):  
Flow Cytometry ◽  
Myelodysplastic Syndromes ◽  
Working Group ◽  
International Consortium

P-30 Prospective validation of the WHO classification of primary myelodysplastic syndromes

2005 ◽  
Vol 29 ◽  
pp. S35
Author(s):  
A. Kuendgen ◽  
S. Knipp ◽  
M. Aivado ◽  
M. Lara ◽  
S. Isa ◽  
...  
Keyword(s):  
Myelodysplastic Syndromes ◽  
Who Classification

Prognostic Factors and Life Expectancy in Myelodysplastic Syndromes Classified According to the WHO Criteria. A Basis for Clinical Decision-Making.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 1430-1430 ◽  
Author(s):  
Luca Malcovati ◽  
Matteo G. Della Porta ◽  
Cristiana Pascutto ◽  
Lucia Malabarba ◽  
Erica Travaglino ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
General Population ◽  
Life Expectancy ◽  
Myelodysplastic Syndromes ◽  
Prognostic Value ◽  
Who Classification ◽  
Cox Regression ◽  
Who Criteria ◽  
Blast Count

Abstract The WHO recently proposed a new classification of myelodysplastic syndromes (MDS) based on uni- or multi-lineage hematopoietic involvement, blast count and cytogenetic features. The aims of this study were to evaluate the prognostic value of the WHO classification, to assess the role of known prognostic factors in MDS within these WHO subgroups, and to estimate mortality rates and life expectancy of the patients in the subgroups. Four hundred and ninety-one consecutive patients with a diagnosis of MDS made at the IRCCS Policlinico San Matteo, University of Pavia Medical School, Italy, between 1992 and 2002 were retrospectively evaluated and reclassified according to the WHO criteria. Cox proportional hazards regression was used to identify the most significant prognostic factors. A standardized mortality ratio (SMR) was calculated to compare the mortality of MDS patients with that of the general population. Overall survival (OS) and leukemia-free survival (LFS) differed significantly between RA and RCMD (P&lt;.001 and P=.01, respectively), but not between RA and MDS with del(5q), or between MDS with or without ringed sideroblasts. There was a significant difference in LFS between RCMD and RAEB-1 (P=.006), and in both OS and LFS between RAEB-1 and -2 (P&lt;.001). Overall, age and gender had significant effects on survival (P&lt;.001), older age and male gender negatively affecting the prognosis. These effects were statistically significant for RA and RCMD patients (P values from.01 to &lt;.001), but not for those with RAEB. The mortality rate of all patients with RA/RARS aged 70 years or older and the male subgroup aged 65-70 years old did not differ from that of the general population. Cox regression analysis with time dependent covariates was applied to evaluate the prognostic value of needing transfusion. Patients with RA/RARS who became transfusion-dependent had a significantly shorter life expectancy than those who did not (P=.01) except for patients aged 70 years or older whose life expectancy was only marginally shorter than that of the general population (SMR=1.90, P=.03). Cox regression showed that IPSS significantly stratified OS and LFS of WHO subgroups (P=.005 and P=.003, respectively). Among IPSS variables, blast count and peripheral cytopenia failed to predict survival within the WHO subgroups, while karyotypic abnormalities, classified according to IPSS, significantly affected OS and LFS of MDS stratified in WHO categories (P=.03 and P=.02, respectively). In conclusion, the new WHO classification of MDS has relevant prognostic value. Cytogenetics is the only independent prognostic factor significantly affecting survival of patients classified into these WHO subgroups. MDS with uni-lineage dysplasia identifies a subset of truly low risk patients, whose survival is significantly affected by demographic characteristics. Patients with refractory anemia who are older than 70 years, as well the males aged between 65 and 70 years, have a life expectancy similar to that of the general population, and only slightly worsened by becoming transfusion-dependent. According to these results, extending curative approaches, such as non-myeloablative transplantation, to these groups of patients does not seem advisable.

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