Dataset Information

Incidence of myelodysplastic syndrome in Japan.

ABSTRACT:

Background

Myelodysplastic syndromes (MDS) are a diverse group of clonal hematopoietic stem cell malignancies that primarily affect the elderly. Although the incidence of MDS in western countries has been well investigated, little is known about the incidence in Asian populations.

Methods

To identify the incidence of MDS in Japan, we used population-based registry data from 1993 to 2008. The data represented 33.1% of the Japanese population. A total of 7995 patients were reported to the registry with newly diagnosed MDS during the study period.

Results

Median age at diagnosis was 76 years. Incidence sharply increased with age, particularly in those aged over 70 years. The most recent crude incidence rate of MDS was 3.8 (95% CI 3.6-4.1) cases per 100 000 for men and 2.4 (95% CI 2.2-2.6) cases per 100 000 for women in 2008. Age-adjusted incidences of MDS in 2008, standardized by the world standard population, were 1.6 and 0.8 cases per 100 000 for men and women, respectively, while incidences standardized by the 1985 Japanese population were 2.5 and 1.2 cases per 100 000 for men and women, respectively.

Conclusions

Our study reveals that some elderly patients suffer from MDS in Japan, but the incidence is lower than in Western countries. In current clinical practice, many elderly MDS patients are treated with supportive therapy based on an incomplete diagnosis, suggesting that the incidence estimated in this study might still be substantially underestimated. Accurate evaluation of the health impact of MDS requires evaluation of the mortality of the disease, continued surveillance, and improvement in the quality of cancer registry data.

SUBMITTER: Chihara D

PROVIDER: S-EPMC4213221 | biostudies-literature | 2014

REPOSITORIES: biostudies-literature

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Publications

Incidence of myelodysplastic syndrome in Japan.

Chihara Dai D Ito Hidemi H Katanoda Kota K Shibata Akiko A Matsuda Tomohiro T Sobue Tomotaka T Matsuo Keitaro K

Journal of epidemiology 20140802 6

<h4>Background</h4>Myelodysplastic syndromes (MDS) are a diverse group of clonal hematopoietic stem cell malignancies that primarily affect the elderly. Although the incidence of MDS in western countries has been well investigated, little is known about the incidence in Asian populations.<h4>Methods</h4>To identify the incidence of MDS in Japan, we used population-based registry data from 1993 to 2008. The data represented 33.1% of the Japanese population. A total of 7995 patients were reported ...[more]

PMID: 25088696

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Project description:BackgroundFerroptosis is a form of iron-dependent non-apoptotic cell death, with characteristics of loss of the activity of the lipid repair enzyme, glutathione (GSH) peroxidase 4 (GPX4), and accumulation of lethal reactive lipid oxygen species. The mechanism of ferroptosis in myelodysplastic syndrome (MDS) is unclear.MethodsCell viability assay, reactive oxygen species (ROS) assay, GSH assay, and GPX activity assay were performed to study the regulation of ferroptosis in MDS cells obtained from MDS patients, the iron overload model mice, and cell lines.ResultsThe growth-inhibitory effect of decitabine could be partially reversed by ferrostatin-1 and iron-chelating agent [desferrioxamine (DFO)] in MDS cell lines. Erastin could increase the cytotoxicity of decitabine on MDS cells. The level of GSH and the activity of GPX4 decreased, whereas the ROS level increased in MDS cells upon treatment with decitabine, which could be reversed by ferrostatin-1. The concentration of hemoglobin in peripheral blood of iron overload mice was negatively correlated with intracellular Fe2+ level and ferritin concentration. Iron overload (IO) led to decreased viability of bone marrow mononuclear cells (BMMNCs), which was negatively correlated with intracellular Fe2+ level. Ferrostatin-1 partially reversed the decline of cell viability in IO groups. The level of GSH and the activity of GPX4 decreased, whereas the ROS level increased in BMMNCs of IO mice. DFO could increase the level of GSH. Ferrostatin-1 and DFO could increase the GPX4 activity of BMMNCs in IO mice. Ferrostatin-1 could significantly reverse the growth-inhibitory effect of decitabine in MDS patients. Decitabine could significantly increase the ROS level in MDS groups, which could be inhibited by ferrostatin-1 or promoted by erastin. Ferrostatin-1 could significantly reverse the inhibitory effect of decitabine on GSH levels in MDS patients. Erastin combined with decitabine could further reduce the GSH level. Erastin could further decrease the activity of GPX4 compared with the decitabine group.ConclusionFerroptosis may account for the main mechanisms of how decitabine induced death of MDS cells. Decitabine-induced ROS raise leads to ferroptosis in MDS cells by decreasing GSH level and GPX4 activity.

Project description:BACKGROUND:Allogeneic hematopoietic stem-cell transplantation is the only curative treatment for patients with myelodysplastic syndrome (MDS). The molecular predictors of disease progression after transplantation are unclear. METHODS:We sequenced bone marrow and skin samples from 90 adults with MDS who underwent allogeneic hematopoietic stem-cell transplantation after a myeloablative or reduced-intensity conditioning regimen. We detected mutations before transplantation using enhanced exome sequencing, and we evaluated mutation clearance by using error-corrected sequencing to genotype mutations in bone marrow samples obtained 30 days after transplantation. In this exploratory study, we evaluated the association of a mutation detected after transplantation with disease progression and survival. RESULTS:Sequencing identified at least one validated somatic mutation before transplantation in 86 of 90 patients (96%); 32 of these patients (37%) had at least one mutation with a maximum variant allele frequency of at least 0.5% (equivalent to 1 heterozygous mutant cell in 100 cells) 30 days after transplantation. Patients with disease progression had mutations with a higher maximum variant allele frequency at 30 days than those who did not (median maximum variant allele frequency, 0.9% vs. 0%; P<0.001). The presence of at least one mutation with a variant allele frequency of at least 0.5% at day 30 was associated with a higher risk of progression (53.1% vs. 13.0%; conditioning regimen-adjusted hazard ratio, 3.86; 95% confidence interval [CI], 1.96 to 7.62; P<0.001) and a lower 1-year rate of progression-free survival than the absence of such a mutation (31.3% vs. 59.3%; conditioning regimen-adjusted hazard ratio for progression or death, 2.22; 95% CI, 1.32 to 3.73; P=0.005). The rate of progression-free survival was lower among patients who had received a reduced-intensity conditioning regimen and had at least one persistent mutation with a variant allele frequency of at least 0.5% at day 30 than among patients with other combinations of conditioning regimen and mutation status (P?0.001). Multivariate analysis confirmed that patients who had a mutation with a variant allele frequency of at least 0.5% detected at day 30 had a higher risk of progression (hazard ratio, 4.48; 95% CI, 2.21 to 9.08; P<0.001) and a lower 1-year rate of progression-free survival than those who did not (hazard ratio for progression or death, 2.39; 95% CI, 1.40 to 4.09; P=0.002). CONCLUSIONS:The risk of disease progression was higher among patients with MDS in whom persistent disease-associated mutations were detected in the bone marrow 30 days after transplantation than among those in whom these mutations were not detected. (Funded by the Leukemia and Lymphoma Society and others.).

Dataset Information

Incidence of myelodysplastic syndrome in Japan.

Background

Methods

Results

Conclusions

Publications

Incidence of myelodysplastic syndrome in Japan.

Similar Datasets

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