Contributions
Abstract: PB1930
Type: Publication Only
Background
Myelodysplastic syndromes (MDS) are heterogeneous disorders defined as clonal diseases involving hematopoietic stem cells and even characterized by cytopenias, with a high risk of leukemic transformation. Morphological analysis of peripheral blood (PB) and marrow aspirates or bone marrow biopsies is the first step that ensures a diagnosis of MDS. Cytogenetic studies are important means of defining different prognostic groups and even of showing how patients are eligible for this or that treatment. We conducted the first study including conventional karyotyping and fluorescent in situ hybridization (FISH) for MDS in our country.
Aims
Our study was aimed to evaluate outcome of MDS regarding IPSS and IPSS-R classification in an emerging country.
Methods
Between January 2012 to December 2016, 101 patients with MDS were consecutively diagnosed. Frequent genetic abnormalities in MDS were screened by R-banding karyotype, metaphase and interphase FISH using a panel including six probes ( 5q-,7q-,20q-, del(17p13), MLL, Inv(3) t(3;3). Patients were stratified into risk groups according to IPSS and IPSS-R scores; survival probabilities were estimated using the Kaplan-Meier method.
Results
Among these 101 pts, 58 were male with a sex ratio = 1, 35; range in age is from 18 years to 94 years with a median of 61, 6 years. Median hemoglobin value was 80 g/L (29-150), more than 60% of patients had severe anemia mainly in the low risk group; the median absolute neutrophil count was 3 G/L (0,060-13, 5), and the median platelet count was 144 G/L (5-659). Median bone marrow blast value was 4 % (0-18). Cases were classed by cytomorphology FAB as RA (N=45), REAB (n=34), RARS (n=16), other (n=6). Classification by WHO 2008 included RCUD (n= 31 of which RA: 18, RT : 10, RN : 3), RCMD (n= 16), RAEB-1 (n=22), RAEB-2 (n= 13), RARS (n= 15), Isolated 5q- (n=4). Among 101 patients, cytogenetic abnormalities by R banding karyotype (n= 84) and FISH (n=101) were found in 41 cases (41%) distributed as single anomaly (n=19), double anomaly (n=5) and complex (n=17). The main cytogenetic abnormalities seen were isolated 5q deletion (n=4), isolated 7q deletion (n=2), isolated 20q deletion (n= 6), isolated trisomy 8 (n=2), 17p13 deletion (n=6), - Y (n=1), complex aberrations ≥ 3 (n=6), complex aberrations ≥ 5 (n=6), complex aberrations ≥7 (n=5), others (n=3). IPSS was assessed in 84 patients: 27 % (low risk), 44 % (intermediate 1), 24 % (intermediate 2), 5 % (high risk). IPSS-R was assessed in 84 patients (18 % very low risk, 30 % low risk, 22,5 % intermediate, 15,5 % High risk, 14 % very High risk). Leukemic transformation into AML occurred in 33% of patients in a median time of 12 months. According to IPSS, the median OS time survival is not reached for low risk group, 41 months (m) for Intermediate 1 risk, 11 m for Intermediate 2 risk, and 4 m for High risk. According to IPSS-R, the median OS time survival is not reached for Very low risk, 43 m for low risk, 24 m for Intermediate risk, 18 m for High risk and 4 m for very high risk.
Conclusion
Our results are in agreement with those previously published regarding demographic features, distribution of recurring cytogenetic abnormalities and prediction of survival. Myelodysplasias are among the most difficult hematological diseases to treat. Treatment of low risk and high risk myelodysplasia are completely different, the last group carrying a great risk of leukemic transformation. For all these reasons, application of the new tools to classify MDS is of a major importance. This is especially true in emerging countries where few therapeutic means are available, hence the need to predict the prognosis of these diseases in order to better target treatments. To the best of our knowledge, it is the first study conducted in our country.
Session topic: 10. Myelodysplastic syndromes - Clinical
Keyword(s): Prognostic groups, Myelodysplasia, Cytogenetic abnormalities
Abstract: PB1930
Type: Publication Only
Background
Myelodysplastic syndromes (MDS) are heterogeneous disorders defined as clonal diseases involving hematopoietic stem cells and even characterized by cytopenias, with a high risk of leukemic transformation. Morphological analysis of peripheral blood (PB) and marrow aspirates or bone marrow biopsies is the first step that ensures a diagnosis of MDS. Cytogenetic studies are important means of defining different prognostic groups and even of showing how patients are eligible for this or that treatment. We conducted the first study including conventional karyotyping and fluorescent in situ hybridization (FISH) for MDS in our country.
Aims
Our study was aimed to evaluate outcome of MDS regarding IPSS and IPSS-R classification in an emerging country.
Methods
Between January 2012 to December 2016, 101 patients with MDS were consecutively diagnosed. Frequent genetic abnormalities in MDS were screened by R-banding karyotype, metaphase and interphase FISH using a panel including six probes ( 5q-,7q-,20q-, del(17p13), MLL, Inv(3) t(3;3). Patients were stratified into risk groups according to IPSS and IPSS-R scores; survival probabilities were estimated using the Kaplan-Meier method.
Results
Among these 101 pts, 58 were male with a sex ratio = 1, 35; range in age is from 18 years to 94 years with a median of 61, 6 years. Median hemoglobin value was 80 g/L (29-150), more than 60% of patients had severe anemia mainly in the low risk group; the median absolute neutrophil count was 3 G/L (0,060-13, 5), and the median platelet count was 144 G/L (5-659). Median bone marrow blast value was 4 % (0-18). Cases were classed by cytomorphology FAB as RA (N=45), REAB (n=34), RARS (n=16), other (n=6). Classification by WHO 2008 included RCUD (n= 31 of which RA: 18, RT : 10, RN : 3), RCMD (n= 16), RAEB-1 (n=22), RAEB-2 (n= 13), RARS (n= 15), Isolated 5q- (n=4). Among 101 patients, cytogenetic abnormalities by R banding karyotype (n= 84) and FISH (n=101) were found in 41 cases (41%) distributed as single anomaly (n=19), double anomaly (n=5) and complex (n=17). The main cytogenetic abnormalities seen were isolated 5q deletion (n=4), isolated 7q deletion (n=2), isolated 20q deletion (n= 6), isolated trisomy 8 (n=2), 17p13 deletion (n=6), - Y (n=1), complex aberrations ≥ 3 (n=6), complex aberrations ≥ 5 (n=6), complex aberrations ≥7 (n=5), others (n=3). IPSS was assessed in 84 patients: 27 % (low risk), 44 % (intermediate 1), 24 % (intermediate 2), 5 % (high risk). IPSS-R was assessed in 84 patients (18 % very low risk, 30 % low risk, 22,5 % intermediate, 15,5 % High risk, 14 % very High risk). Leukemic transformation into AML occurred in 33% of patients in a median time of 12 months. According to IPSS, the median OS time survival is not reached for low risk group, 41 months (m) for Intermediate 1 risk, 11 m for Intermediate 2 risk, and 4 m for High risk. According to IPSS-R, the median OS time survival is not reached for Very low risk, 43 m for low risk, 24 m for Intermediate risk, 18 m for High risk and 4 m for very high risk.
Conclusion
Our results are in agreement with those previously published regarding demographic features, distribution of recurring cytogenetic abnormalities and prediction of survival. Myelodysplasias are among the most difficult hematological diseases to treat. Treatment of low risk and high risk myelodysplasia are completely different, the last group carrying a great risk of leukemic transformation. For all these reasons, application of the new tools to classify MDS is of a major importance. This is especially true in emerging countries where few therapeutic means are available, hence the need to predict the prognosis of these diseases in order to better target treatments. To the best of our knowledge, it is the first study conducted in our country.
Session topic: 10. Myelodysplastic syndromes - Clinical
Keyword(s): Prognostic groups, Myelodysplasia, Cytogenetic abnormalities