scholarly journals The revised who classification of myelodysplastic syndromes – What has changed?

Pathology ◽  
2020 ◽  
Vol 52 ◽  
pp. S37-S38
Author(s):  
Gina Zini
Keyword(s):  
Myelodysplastic Syndromes ◽  
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.

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.

Hematological Characteristics of Patients with Myelodysplastic Syndromes in the Light of WHO 2008 Proposals.

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 1658-1658
Author(s):  
Kathrin Nachtkamp ◽  
Andrea Kuendgen ◽  
Barbara Hildebrandt ◽  
Norbert Gattermann ◽  
Rainer Haas ◽  
...  
Keyword(s):  
Myelodysplastic Syndromes ◽  
Who Classification ◽  
Human Genetics ◽  
Risk Groups ◽  
Hemoglobin Level ◽  
Strongly Correlated ◽  
New Classification ◽  
Characteristic Features ◽  
Who 2008

Abstract Myelodysplastic syndromes have been reclassified by new WHO proposals published in 2008. So far, there are no data on haematological features of the newly composed subtypes. We analyzed 2063 patients with MDS diagnosed according to the WHO 2008 classification. Patients were characterized by their initial haematological features. We especially payed attention towards isolated cytopeniae with regard to the new classification of MDS subcategories. Analyses were performed by the use of the MDS registry Duesseldorf and included hemoglobin level, platelets, leukocytes and karyotype (available in 1091 patients) as well as age, gender, IPSS and WPSS at the time of diagnosis. All items have been assessed in our haematological laboratory and in the department of human genetics, respectively. The table shows characteristic features of MDS patients according to their WHO subtype. The patients’ median age did not differ substantially. There was a preponderance of females in the del(5q), RARS and RC group. Median hemoglobin level ranked lowest in the del(5q) and RAEB II group. Median thrombocyte, leukocyte as well as granulocyte level progressively decreased with more advanced WHO risk groups. Chromosomal aberrations were more frequent with increasing medullary blasts and degree of dysplasia. Likewise, patients with these characteristics were categorized into more advanced WPSS subtypes. About 90% of all patients were anemic, that is patients with a hemoglobin level of under 12 g/dl. Leukocytopenia as well as thrombocytopenia were more severe with increasing medullary blast counts. Of interest, 46% of RC patients showed a leukocytopenia (&lt;4000/μl) and 37% had thrombocytopenia (&lt;150.000/μl). 26% of this group showed an isolated anemia, whereas only 1–3% of the patients presented with an isolated leukocytopenia or thrombocytopenia. The majority of MDS patients presents with bicytopenia, regardless of subtypes. The degree of hematopoietic insufficiency is strongly correlated with the amount of medullary blasts, but as well with the degree of dysplasia in blood and marrow. An inclusion of the number of cytopenia into the WHO classification would lead to an alteration of subcategories and result in a shift towards a higher percentage of multilineage MDS. RC RARS MDS with del(5q) RCMD RAEB I RAEB II Frequency 184 162 68 950 306 393 Age 70 70 64 72 69 67 Gender (%) M 48 48 35 55 58 56 W 52 52 65 45 42 44 Hemoglobin (g/dl) 9,5 9,5 9 9,2 9,2 9 Thrombocytes (1000/μl) 124 293 284 146 96 80 Leukocytes (/μl) 4800 5500 4300 3900 3100 2800 Granulocytes 1950 3200 2400 2200 1400 1200 Chromosomal Low 70 87 96 63 57 51 risk by IPSS Intermediate 19 10 2 18 16 19 (%) High 11 3 2 19 27 30 Initial IPSS (%) 0 48 83 72 34 0 0 1 42 14 26 53 2 7 2 10 3 2 13 55 50 3 0 0 0 0 40 43 Initial WPSS 0 48 73 40 30 0 0 (%) 1 30 27 60 40 0 0 2 22 0 0 30 33 0 3 0 0 0 0 50 59 4 0 0 0 0 18 41 Cytopenia (%) Anemia (&lt;12 g/dl) 85 97 98 89 86 90 Leukopenia 46 18 39 50 64 89 (&lt;4000/μl) Thrombocytopenia 37 3 8 35 52 80 (&lt;150.000/μl) Isolated (%) Anemia 26 78 51 27 14 7 Leukocytopenia 1,5 0 1 1 2 1 Thrombocytopenia 3 0 0 2 3 2 Pancytopenia 22 3 6 24 35 44 (%) Survival (mths.) 561 69 80 36 19 10 AML after 3 3 15 15 32 63 3 yrs. (%)

scholarly journals Correspondence Re: Brunning R: Proposed Who Classification of Acute Myeloid Leukemia and Myelodysplastic Syndromes. Mod Pathol 1999;1:102–4.

2000 ◽  
Vol 13 (1) ◽  
pp. 101-102 ◽  
Author(s):  
Fermina M Mazzella ◽  
James D Cotelingam ◽  
Areta Kowal-Vern ◽  
M Atef Shrit ◽  
James T Rector ◽  
...  
Keyword(s):  
Acute Myeloid Leukemia ◽  
Myelodysplastic Syndromes ◽  
Myeloid Leukemia ◽  
Who Classification ◽  
Acute Myeloid

scholarly journals Reclassifying myelodysplastic syndromes: what's where in the new WHO and why

Hematology ◽  
2015 ◽  
Vol 2015 (1) ◽  
pp. 294-298 ◽  
Author(s):  
Daniel A. Arber ◽  
Robert P. Hasserjian
Keyword(s):  
Advisory Committee ◽  
Diagnostic Criteria ◽  
Myelodysplastic Syndromes ◽  
Who Classification ◽  
Prognostic Significance ◽  
Gene Mutations ◽  
Refractory Anemia ◽  
Ring Sideroblasts ◽  
Actual Publication

Abstract A revision to the 4th edition of the WHO Classification of myelodysplastic syndromes (MDSs), originally published in 2008, is expected in mid-2016. Based on recommendations of a Clinical Advisory Committee, the revision will aim to incorporate new discoveries in MDS that impact existing disease categories. Although the basic diagnostic principles of the WHO classification remain unchanged, several changes to the classification are proposed. All revisions are considered preliminary until the actual publication of the monograph and online document. Proposals for change include abandoning the routine use of “refractory anemia/cytopenia” in the various disease names, including the prognostic significance of gene mutations in MDS, revising the diagnostic criteria for MDS entities with ring sideroblasts based on the detection of SF3B1 mutations, modifying the cytogenetic criteria for MDS with isolated del(5q), reclassifying most cases of the erythroid/myeloid type of acute erythroleukemia, and recognizing the familial link in some cases of MDS. This review will provide details of the major proposed changes as well as rationale for the revisions.

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