CLINICAL AND EPIDEMIOLOGICAL CHARACTERISTICS OF MYELODYSPLASTIC SYNDROMES (MDS) IN ADULTS ACCORDING TO THE DATA OF MUNICIPAL HEMATOLOGY DEPARTMENTS IN MOSCOW, RUSSIA
(Abstract release date: 05/19/16)
EHA Library. Semochkin S. 06/09/16; 134826; PB1926
Prof. Dr. Sergey Semochkin
Contributions
Contributions
Abstract
Abstract: PB1926
Type: Publication Only
Background
The myelodysplastic syndromes (MDS) are a distinct group of clonal disorders of hematopoietic stem or progenitor cells characterized by ineffective hematopoiesis, peripheral cytopenias, abnormal dysplastic cell morphology, and potential for clonal evolution to secondary acute myeloid leukemia (AML). AML is eventually diagnosed in up to 30-40% of MDS cases. Population-based registry data in the USA and Europe indicate an incidence of MDS in western countries of around 3-5 cases per 100.000 person-years with male predominance. The epidemiology of MDS has been carefully investigated in western countries while exact data on the epidemiology of MDS in Russia are absent.
Aims
To study the registered incidence of MDS in adults in the city of Moscow, to evaluate methods of diagnosis confirmation and choice of therapy in the system of Health Care.
Methods
The observational study included adult patients with newly diagnosed MDS in 2010, who were residents of Moscow. Evaluation of overall survival (OS) conducted as of September 2014.
Results
A total of 201 (male – 110, female – 118) adult patients were reported to the system of Moscow Health Care with newly diagnosed MDS in 2010. Median age at diagnosis was 71.5 years (range, 23.9-93.7). The incidence rate of MDS was 2.0 cases per 100.000 persons per year in the general adult population. Incidence accurately increased with age (Fig. 1). The maximum incidence 18.1 cases per 100.000 men was registered in the age over ≥ 85 years and 9.8 cases per 100.000 women in age 75-79 years. Most frequent morphological types of MDS distributed as follows: refractory anemia (RA) – 33.8%, refractory anemia with ring sideroblasts (RARS) – 8.4%, refractory cytopenia with multilineage dysplasia (RCMD) – 12.0%, refractory anemia with excess blasts-1 (RAEB-1) – 12.9% и RAEB-2 – 21.9%. Cytogenetic variants of MDS assessed only in 36 (17.9%) patients. All patients divided into 5 groups depending on the type of first-line therapy: 69 patients treated with epoetin alfa or beta (rHuEpo) as monotherapy; 20 – low-dose ara-C (LDAC); 12 – hypomethylating agents (azacitidine, decitabine); 60 – symptomatic (red cell transfusion for low-risk MDS) and 38 - palliative care (elderly and weakened high-risk patients). Two patients with 5q- syndrome treated with lenalidomide. With a median follow-up for survivors 46 months 4-year overall survival (OS) for all patients was 34.8 ± 13.4% (median 24.3 months). Early mortality over the first 60 days was 5.0%. rHuEpo used primarily in low-risk patients: 4-year OS 60.7 ± 5.9%. Hypomethylation agents and LDAC applied in advanced MDS morphological variants: 4-year OS 25.0 ± 12.5% (median 21.1 months) and 15.0 ± 8.0% (15.3 months) respectively. Depending on the morphological variant of the 4-year OS was the following: 5q- syndrome – 57.1 ± 18.7% (median › 46 months); RA – 50.8 ± 6.1% (35.7); RARS – 35.3 ± 11.6% (32.8); RAEB-1 - 32.8 ± 9.4% (24.7) and RAEB-2 – 5.7 ± 3.7% (15.3).
Conclusion
The incidence of MDS in Moscow, Russia is 1.5-2 times lower than in Europe and the United States. Current diagnostic standards under the mandatory health insurance does not include genetic and molecular studies. Implementation a cytogenetic study and FISH in MDS typical chromosomal abnormalities in all patients with unexplained cytopenias will improve the diagnostic level.
Session topic: E-poster
Keyword(s): Epidemiology, Myelodysplasia, Treatment
Type: Publication Only
Background
The myelodysplastic syndromes (MDS) are a distinct group of clonal disorders of hematopoietic stem or progenitor cells characterized by ineffective hematopoiesis, peripheral cytopenias, abnormal dysplastic cell morphology, and potential for clonal evolution to secondary acute myeloid leukemia (AML). AML is eventually diagnosed in up to 30-40% of MDS cases. Population-based registry data in the USA and Europe indicate an incidence of MDS in western countries of around 3-5 cases per 100.000 person-years with male predominance. The epidemiology of MDS has been carefully investigated in western countries while exact data on the epidemiology of MDS in Russia are absent.
Aims
To study the registered incidence of MDS in adults in the city of Moscow, to evaluate methods of diagnosis confirmation and choice of therapy in the system of Health Care.
Methods
The observational study included adult patients with newly diagnosed MDS in 2010, who were residents of Moscow. Evaluation of overall survival (OS) conducted as of September 2014.
Results
A total of 201 (male – 110, female – 118) adult patients were reported to the system of Moscow Health Care with newly diagnosed MDS in 2010. Median age at diagnosis was 71.5 years (range, 23.9-93.7). The incidence rate of MDS was 2.0 cases per 100.000 persons per year in the general adult population. Incidence accurately increased with age (Fig. 1). The maximum incidence 18.1 cases per 100.000 men was registered in the age over ≥ 85 years and 9.8 cases per 100.000 women in age 75-79 years. Most frequent morphological types of MDS distributed as follows: refractory anemia (RA) – 33.8%, refractory anemia with ring sideroblasts (RARS) – 8.4%, refractory cytopenia with multilineage dysplasia (RCMD) – 12.0%, refractory anemia with excess blasts-1 (RAEB-1) – 12.9% и RAEB-2 – 21.9%. Cytogenetic variants of MDS assessed only in 36 (17.9%) patients. All patients divided into 5 groups depending on the type of first-line therapy: 69 patients treated with epoetin alfa or beta (rHuEpo) as monotherapy; 20 – low-dose ara-C (LDAC); 12 – hypomethylating agents (azacitidine, decitabine); 60 – symptomatic (red cell transfusion for low-risk MDS) and 38 - palliative care (elderly and weakened high-risk patients). Two patients with 5q- syndrome treated with lenalidomide. With a median follow-up for survivors 46 months 4-year overall survival (OS) for all patients was 34.8 ± 13.4% (median 24.3 months). Early mortality over the first 60 days was 5.0%. rHuEpo used primarily in low-risk patients: 4-year OS 60.7 ± 5.9%. Hypomethylation agents and LDAC applied in advanced MDS morphological variants: 4-year OS 25.0 ± 12.5% (median 21.1 months) and 15.0 ± 8.0% (15.3 months) respectively. Depending on the morphological variant of the 4-year OS was the following: 5q- syndrome – 57.1 ± 18.7% (median › 46 months); RA – 50.8 ± 6.1% (35.7); RARS – 35.3 ± 11.6% (32.8); RAEB-1 - 32.8 ± 9.4% (24.7) and RAEB-2 – 5.7 ± 3.7% (15.3).
Conclusion
The incidence of MDS in Moscow, Russia is 1.5-2 times lower than in Europe and the United States. Current diagnostic standards under the mandatory health insurance does not include genetic and molecular studies. Implementation a cytogenetic study and FISH in MDS typical chromosomal abnormalities in all patients with unexplained cytopenias will improve the diagnostic level.
Session topic: E-poster
Keyword(s): Epidemiology, Myelodysplasia, Treatment
Abstract: PB1926
Type: Publication Only
Background
The myelodysplastic syndromes (MDS) are a distinct group of clonal disorders of hematopoietic stem or progenitor cells characterized by ineffective hematopoiesis, peripheral cytopenias, abnormal dysplastic cell morphology, and potential for clonal evolution to secondary acute myeloid leukemia (AML). AML is eventually diagnosed in up to 30-40% of MDS cases. Population-based registry data in the USA and Europe indicate an incidence of MDS in western countries of around 3-5 cases per 100.000 person-years with male predominance. The epidemiology of MDS has been carefully investigated in western countries while exact data on the epidemiology of MDS in Russia are absent.
Aims
To study the registered incidence of MDS in adults in the city of Moscow, to evaluate methods of diagnosis confirmation and choice of therapy in the system of Health Care.
Methods
The observational study included adult patients with newly diagnosed MDS in 2010, who were residents of Moscow. Evaluation of overall survival (OS) conducted as of September 2014.
Results
A total of 201 (male – 110, female – 118) adult patients were reported to the system of Moscow Health Care with newly diagnosed MDS in 2010. Median age at diagnosis was 71.5 years (range, 23.9-93.7). The incidence rate of MDS was 2.0 cases per 100.000 persons per year in the general adult population. Incidence accurately increased with age (Fig. 1). The maximum incidence 18.1 cases per 100.000 men was registered in the age over ≥ 85 years and 9.8 cases per 100.000 women in age 75-79 years. Most frequent morphological types of MDS distributed as follows: refractory anemia (RA) – 33.8%, refractory anemia with ring sideroblasts (RARS) – 8.4%, refractory cytopenia with multilineage dysplasia (RCMD) – 12.0%, refractory anemia with excess blasts-1 (RAEB-1) – 12.9% и RAEB-2 – 21.9%. Cytogenetic variants of MDS assessed only in 36 (17.9%) patients. All patients divided into 5 groups depending on the type of first-line therapy: 69 patients treated with epoetin alfa or beta (rHuEpo) as monotherapy; 20 – low-dose ara-C (LDAC); 12 – hypomethylating agents (azacitidine, decitabine); 60 – symptomatic (red cell transfusion for low-risk MDS) and 38 - palliative care (elderly and weakened high-risk patients). Two patients with 5q- syndrome treated with lenalidomide. With a median follow-up for survivors 46 months 4-year overall survival (OS) for all patients was 34.8 ± 13.4% (median 24.3 months). Early mortality over the first 60 days was 5.0%. rHuEpo used primarily in low-risk patients: 4-year OS 60.7 ± 5.9%. Hypomethylation agents and LDAC applied in advanced MDS morphological variants: 4-year OS 25.0 ± 12.5% (median 21.1 months) and 15.0 ± 8.0% (15.3 months) respectively. Depending on the morphological variant of the 4-year OS was the following: 5q- syndrome – 57.1 ± 18.7% (median › 46 months); RA – 50.8 ± 6.1% (35.7); RARS – 35.3 ± 11.6% (32.8); RAEB-1 - 32.8 ± 9.4% (24.7) and RAEB-2 – 5.7 ± 3.7% (15.3).
Conclusion
The incidence of MDS in Moscow, Russia is 1.5-2 times lower than in Europe and the United States. Current diagnostic standards under the mandatory health insurance does not include genetic and molecular studies. Implementation a cytogenetic study and FISH in MDS typical chromosomal abnormalities in all patients with unexplained cytopenias will improve the diagnostic level.
Session topic: E-poster
Keyword(s): Epidemiology, Myelodysplasia, Treatment
Type: Publication Only
Background
The myelodysplastic syndromes (MDS) are a distinct group of clonal disorders of hematopoietic stem or progenitor cells characterized by ineffective hematopoiesis, peripheral cytopenias, abnormal dysplastic cell morphology, and potential for clonal evolution to secondary acute myeloid leukemia (AML). AML is eventually diagnosed in up to 30-40% of MDS cases. Population-based registry data in the USA and Europe indicate an incidence of MDS in western countries of around 3-5 cases per 100.000 person-years with male predominance. The epidemiology of MDS has been carefully investigated in western countries while exact data on the epidemiology of MDS in Russia are absent.
Aims
To study the registered incidence of MDS in adults in the city of Moscow, to evaluate methods of diagnosis confirmation and choice of therapy in the system of Health Care.
Methods
The observational study included adult patients with newly diagnosed MDS in 2010, who were residents of Moscow. Evaluation of overall survival (OS) conducted as of September 2014.
Results
A total of 201 (male – 110, female – 118) adult patients were reported to the system of Moscow Health Care with newly diagnosed MDS in 2010. Median age at diagnosis was 71.5 years (range, 23.9-93.7). The incidence rate of MDS was 2.0 cases per 100.000 persons per year in the general adult population. Incidence accurately increased with age (Fig. 1). The maximum incidence 18.1 cases per 100.000 men was registered in the age over ≥ 85 years and 9.8 cases per 100.000 women in age 75-79 years. Most frequent morphological types of MDS distributed as follows: refractory anemia (RA) – 33.8%, refractory anemia with ring sideroblasts (RARS) – 8.4%, refractory cytopenia with multilineage dysplasia (RCMD) – 12.0%, refractory anemia with excess blasts-1 (RAEB-1) – 12.9% и RAEB-2 – 21.9%. Cytogenetic variants of MDS assessed only in 36 (17.9%) patients. All patients divided into 5 groups depending on the type of first-line therapy: 69 patients treated with epoetin alfa or beta (rHuEpo) as monotherapy; 20 – low-dose ara-C (LDAC); 12 – hypomethylating agents (azacitidine, decitabine); 60 – symptomatic (red cell transfusion for low-risk MDS) and 38 - palliative care (elderly and weakened high-risk patients). Two patients with 5q- syndrome treated with lenalidomide. With a median follow-up for survivors 46 months 4-year overall survival (OS) for all patients was 34.8 ± 13.4% (median 24.3 months). Early mortality over the first 60 days was 5.0%. rHuEpo used primarily in low-risk patients: 4-year OS 60.7 ± 5.9%. Hypomethylation agents and LDAC applied in advanced MDS morphological variants: 4-year OS 25.0 ± 12.5% (median 21.1 months) and 15.0 ± 8.0% (15.3 months) respectively. Depending on the morphological variant of the 4-year OS was the following: 5q- syndrome – 57.1 ± 18.7% (median › 46 months); RA – 50.8 ± 6.1% (35.7); RARS – 35.3 ± 11.6% (32.8); RAEB-1 - 32.8 ± 9.4% (24.7) and RAEB-2 – 5.7 ± 3.7% (15.3).
Conclusion
The incidence of MDS in Moscow, Russia is 1.5-2 times lower than in Europe and the United States. Current diagnostic standards under the mandatory health insurance does not include genetic and molecular studies. Implementation a cytogenetic study and FISH in MDS typical chromosomal abnormalities in all patients with unexplained cytopenias will improve the diagnostic level.
Session topic: E-poster
Keyword(s): Epidemiology, Myelodysplasia, Treatment
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