World Health Organization and International Prognostic Scoring System: The Limitations of Current Classification Systems in Assessing Prognosis and Determining Appropriate Therapy in Myelodysplastic Syndromes

2008 ◽  
Vol 45 (1) ◽  
pp. 3-7 ◽  
Author(s):  
Charles A. Schiffer
Keyword(s):  
World Health Organization ◽  
Myelodysplastic Syndromes ◽  
Scoring System ◽  
Classification Systems ◽  
World Health ◽  
International Prognostic Scoring System ◽  
Current Classification ◽  
Appropriate Therapy ◽  
Health Organization

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 Ανοσοφαινοτυπική και λειτουργική μελέτη αρχέγονων αιμοποιητικών κυττάρων στο μυελό των οστών ασθενών με πρωτοπαθές μυελοδυσπλαστικό σύνδρομο

2018 ◽  
Author(s):  
Νικόλαος Γαρδίκας
Keyword(s):  
World Health Organization ◽  
Scoring System ◽  
De Novo ◽  
World Health ◽  
International Prognostic Scoring System ◽  
Health Organization

Τα Μυελοδυσπλαστικά σύνδρομα (ΜΔΣ) αποτελούν κλωνικές διαταραχές του αρχέγονου αιμοποιητικού κυττάρου με άγνωστη αιτιολογία. Η Πολυπαραμετρική κυτταρομετρία ροής (ΠΚΡ) έχει διερευνηθεί για τη συμβολή της στη διάγνωση και πρόγνωση των ΜΔΣ. Στην παρούσα διατριβή εφαρμόστηκε ΠΚΡ πενταπλού φθορισμού σε δείγματα μυελού οστών 47 ασθενών με de-novo ΜΔΣ που δεν είχαν λάβει θεραπεία και 27 δείγματα από υγιείς μάρτυρες. Οι ασθενείς ταξινομήθηκαν με βάση τον Παγκόσμιο Οργανισμό Υγείας (World Health Organization 2008, WHO), το Διεθνές σύστημα πρόγνωσης (International Prognostic Scoring System, IPSS) και το Αναθεωρημένο σύστημα πρόγνωσης (Revised IPSS, IPSS-R). Παρατηρήθηκε στατιστικώς σημαντική αύξηση των ποσοστών των CD133+/CD90-CD45weak, CD117+/TdT-CD45weak και CD33+/MPO- πρόδρομων κυττάρων κοκκιώδους σειράς (Neutrophil Precursor, NP) στα CD34+ κύτταρα, όπως και στατιστικώς σημαντική ελάττωση των πρόδρομων κυττάρων λεμφικής και ερυθράς σειράς, στο σύνολο των ασθενών με ΜΔΣ σε σχέση με την ομάδα των υγιών μαρτύρων. Ένα νέο σύστημα βαθμολόγησης βασισμένο σε αυτά τα ευρήματα μπορεί να συμβάλει στη διάκριση των ασθενών με ΜΔΣ χαμηλότερου κινδύνου, συμπεριλαμβανομένων και των ασθενών με φυσιολογικό καρυότυπο (υποομάδα ΜΔΣ που διαγιγνώσκεται δύσκολα). Επιπλέον, η αύξηση του ποσοστού της απόπτωσης των CD34+/CD117+ κυττάρων χαρακτηρίστηκε ως ανεξάρτητος δείκτης καλής πρόγνωσης για την εκτροπή των ασθενών σε AML και για τη συνολική διάρκεια επιβίωσής τους. Ένα νέο σύστημα βαθμολόγησης βασισμένο στην έκφραση των δεικτών των CD34+ κυττάρων (ως ανεξάρτητο ή σε συνδυασμό με το σύστημα βαθμολόγησης Ogata) μπορεί να διακρίνει τους ασθενείς με ΜΔΣ χαμηλότερου κινδύνου, συμπεριλαμβανομένων των ασθενών με φυσιολογικό καρυότυπο, από την ομάδα υγιών μαρτύρων.

Retrospective Analysis of Patients with Refractory Cytopenia with Unilineage Dysplasia.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4847-4847
Author(s):  
Yuchun Wang ◽  
Xin Du ◽  
Suxia Geng
Keyword(s):  
Myelodysplastic Syndromes ◽  
Who Classification ◽  
Conflicts Of Interest ◽  
Major Change ◽  
Classification Systems ◽  
World Health ◽  
Refractory Anemia ◽  
Prognostic Information ◽  
Prognostic Assessment ◽  
Health Organization

Abstract Abstract 4847 Background Myelodysplastic syndromes (MDS) are a group of clonal heterogeneous bone marrow stem cell disorders characterized by dysplastic hypercellular marrows with peripheral cytopenias. Accurate diagnosis and classification are essential for subgroup identification and prognostic assessment of patients with MDS. Classification systems such as the World Health Organization (WHO) classification are widely used but do not always provide sufficient prognostic information. So WHO improved the classification systems last year(2008). The major change was that refractory anemia (RA) was substituted by refractory cytopenia with unilineage dysplasia (RCUD). The aim of this study was to apply the new WHO classification to re-evaluate MDS with RA. Methods MDS patients with RA diagnosed between 2000 and 2008 were retrospectively reclassified with WHO criteria (2008). Results According to the new WHO classification, RCUD is intended to encompass those myelodysplastic syndromes (MDS) which present with a refractory cytopenia with unilineage dysplasia and includes refractory anemia (RA), refractory neutropenia (RN) and refractory thrombocytopenia (RT), so in 34 cases diagnosed as RA, 12 cases (12/34,35%) still pertained to RA, 19 cases (56% ) to RN and 3 (9%) cases to RT. IPSS showed 94% of RCUD cases categorized as low or intermediate-I risk. The median survival of patients with RCUD was 84 months. Conclusions RA is the main subtypes of RCUD. Understanding the clinical features of RCUD accurately is helpful to determine the right subtype of RCUD. The new WHO classification system was improved to provide better diagnostic criteria. 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.

Myelodysplastic Syndromes in Children: A Critical Review of Issues in the Diagnosis and Classification of 887 Cases From 13 Published Series

2007 ◽  
Vol 131 (7) ◽  
pp. 1110-1116
Author(s):  
M. Tarek Elghetany
Keyword(s):  
World Health Organization ◽  
Myelodysplastic Syndromes ◽  
De Novo ◽  
English Literature ◽  
World Health ◽  
Inherited Disorders ◽  
World Health Organization Classification ◽  
Health Organization ◽  
The Impact

Abstract Context.—Pediatric myelodysplastic syndromes (MDSs) are uncommon disorders, which may be difficult to diagnose, particularly in the absence of increased blasts. Pediatric MDSs have several unique features including their association with inherited/constitutional disorders in approximately one third of patients. The classification of pediatric MDSs has undergone significant evolution in the past 20 years. Objective.—To critically review existing classifications of pediatric MDSs and to evaluate their applicability on previously published large series. Data Sources.—Previously published pediatric MDS series containing more than 10 patients from the English literature between 1982 and 2005. Conclusions.—Data were available on 887 patients from 13 published series. Most cases (68.7%) were idiopathic/ de novo, 23.9% were associated with constitutional/inherited disorder, and 7.4% were therapy related. Approximately 10% of cases could not be classified by the French-American-British classification. Eighty-seven percent of unclassified cases were appropriately classified using the World Health Organization classification (2001), whereas 96% of them were classified with the modified World Health Organization classification for pediatric MDSs (2003). The impact of cytogenetics and constitutional/inherited disorders on the biology and outcome of the disease needs to be studied further.

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