Proposals for the classification of the myelodysplastic syndromes

Bennett, John M.; Catovsky, Daniel; Mt, Daniel; Flandrin, Georges; Galton, D. A. G.; Gralnick, Harvey R.; Sultan, C.

doi:10.1111/j.1365-2141.1982.tb08475.x

Cited by 3,374 publications

(764 citation statements)

References 6 publications

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“…The French, American, and British (FAB) classification has been employed in order to classify MDS as low-, intermediate-, or high-risk MDS, according to the International Prognostic Scoring System (IPSS) (4,5). The IPSS is based on certain criteria such as the number of cytopenias, cytogenetic profile, and the percentage of blasts in the bone marrow, and allows the classification of MDS patients into 1 of 4 prognostic categories: low risk, intermediate 1 risk, intermediate 2 risk, and high risk.…”

Section: Methodsmentioning

confidence: 99%

Systemic and immune manifestations in myelodysplasia: A multicenter retrospective study

Hollanda¹,

Beucher²,

Henrion³

et al. 2011

Arthritis Care & Research

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Objective. The presence of systemic and/or immune manifestations in myelodysplasia has been currently reported. The influence of these manifestations on the natural outcome of myelodysplastic syndrome has to be considered. We present a multicenter retrospective study (2002-2009) of patients with myelodysplastic syndrome disclosing systemic and/or immune manifestations. Methods. Forty-six patients with myelodysplasia presenting with systemic and/or immune manifestations were compared in terms of survival with 189 patients with myelodysplasia lacking these features. Results. The clinical picture in these cases consisted of fever (13%), arthralgia or arthritis (13%), and cutaneous manifestations (67%). Four cases of systemic vasculitis have been reported in our series, and they have a worse prognosis. Immune anomalies were recorded in 29% of the cases, and the presence of cryoglobulins was also associated with a worse prognosis. Conclusion. A difference in survival between patients with myelodysplastic syndrome with systemic manifestations and patients lacking these manifestations has been observed in the presence of systemic vasculitis and/or cryoglobulins.

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Section: Methodsmentioning

confidence: 99%

Systemic and immune manifestations in myelodysplasia: A multicenter retrospective study

Hollanda¹,

Beucher²,

Henrion³

et al. 2011

Arthritis Care & Research

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“…Myelodysplastic syndromes (MDSs) are a group of stem cell disorders characterized by ine ective hematopoiesis with trilineage dysplasia and often correlated with transformation into acute myeloid leukemia (AML) (Bennett et al, 1982;Koe er, 1986). MDS patients have peripheral cytopenias despite frequently having normo-or hypercellular bone marrows (BMs).…”

Section: Introductionmentioning

confidence: 99%

Apoptosis induced by the myelodysplastic syndrome-associated NPM-MLF1 chimeric protein

1999

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The NPM-MLF1 chimeric protein is produced by the t(3;5)(q25.1;q34) chromosomal translocation, which is associated with myelodysplastic syndrome (MDS) prior to progression into acute myeloid leukemia (AML). Here we report that K562 human leukemia cells ectopically expressing NPM-MLF1, but not those with wild-type MLF1, were gradually eliminated from the culture by undergoing apoptosis. NIH3T3 mouse ®broblasts engineered to overexpress NPM-MLF1 grew normally but serum deprivation triggered apoptotic cell death with slower kinetics than did other well-known apoptotic inducers such as c-Myc or E2F-1. Quantitative analysis of apoptotic induction con®rmed that, neither NPM nor MLF1, but the NPM-MLF1 fusion protein was able to induce apoptosis. Analyses using a variety of deletion mutants of NPM-MLF1 revealed that induction of apoptosis required the N-terminal domain of MLF1 and the NPM domain containing nuclear localization signal and that removal of the NPM dimerization domain markedly impaired the ability to induce apoptosis. Co-expression of Bcl-2 rescued NIH3T3 ®broblasts from NPM-MLF1-mediated cell death without a ecting the expression level or the subcellular localization of NPM-MLF1 and enabled cells to progress into S phase in low serum. These ®ndings provide an NPM-MLF1-mediated novel mechanism of apoptotic induction and imply that NPM-MLF1 in collaboration with anti-apoptotic oncoproteins may play an important role in multi-step progression from MDS to AML.

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“…The diagnosis and further classification of MDS was according to the criteria suggested by the FrenchAmerican-British (FAB) Co-operative Group. 2 …”

Section: Methodsmentioning

confidence: 99%

Myelodysplastic syndromes associated with 11q23 abnormalities

et al. 1998

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Most hematological neoplasms associated with 11q23 chromosomal rearrangement have been cases of acute lymphoblastic or acute myeloid leukemia. Although cases of myelodysplastic syndrome (MDS) are a minority, these syndromes should also be recognized as part of the spectrum of hematological disorders associated with 11q23 rearrangement. In this series of 550 patients with such rearrangement there were 28 (5.1%) who presented with MDS. A further five patients had a history of MDS but had evolved to AML by the time cytogenetic analysis was first undertaken. There were thus a total of 33 patients (6.0%) with an 11q23 abnormality whose initial presentation was as MDS. Of these 33 patients, a quarter (seven patients) were cases of secondary (therapy-related) MDS. Complex karyotypes and other poor prognosis chromosomal abnormalities such as −7 and 7q− were common and were not confined to secondary cases. The likelihood of presentation as MDS differed between different cytogenetic categories. The 28 patients in whom cytogenetic analysis was performed at presentation as MDS showed a wide age distribution, from 1 to 82 years; there were four children, two of 1 year of age. All FAB types of MDS were represented. Median survival was only 19.1 months. Leukemic transformation occurred in five patients including one case of transformation to a biphenotypic M5a/T-lineage acute leukemia.

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Proposals for the classification of the myelodysplastic syndromes

Cited by 3,374 publications

References 6 publications

Systemic and immune manifestations in myelodysplasia: A multicenter retrospective study

Systemic and immune manifestations in myelodysplasia: A multicenter retrospective study

Apoptosis induced by the myelodysplastic syndrome-associated NPM-MLF1 chimeric protein

Myelodysplastic syndromes associated with 11q23 abnormalities

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