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A CLINICAL STUDY ON MYELODYSPLASTIC SYNDROMES
Author(s): ,
Adela Perolla
Affiliations:
Hematology,UHC ' Mother Teresa',Tirana,Albania
,
Alma Cili
Affiliations:
Hematology,UHC ' Mother Teresa',Tirana,Albania
,
Elsuarta Çalliku
Affiliations:
Hematology,UHC ' Mother Teresa',Tirana,Albania
,
Polikron Pulluqi
Affiliations:
Hematology,UHC ' Mother Teresa',Tirana,Albania
,
Tatjana Caja
Affiliations:
Hematology,UHC ' Mother Teresa',Tirana,Albania
Arben Ivanaj
Affiliations:
Hematology,UHC ' Mother Teresa',Tirana,Albania
EHA Library. Perolla A. 06/09/21; 324303; PB1628
Dr. Adela Perolla
Dr. Adela Perolla
Contributions
Abstract

Abstract: PB1628

Type: Publication Only

Session title: Myelodysplastic syndromes - Clinical

Background
Myelodysplastic Syndromes (MDS) compound a  heterogeneous group of malignant  hematological diseases presenting an ineffective hematopoiesis and a tendency of evolution to acute myeloblastic leukemia. The natural course of MDS is very different among the subgroups emphasizing the importance of the correct stratification / subclassification of those patients to better diagnose and treat them.  

Aims
To study the incidence of Myelodysplastic Syndromes (MDS), risk factors, concomitant diseases, genetic abnormalities at the moment of the diagnosis.Our study aims to evaluate the median survival and the overall survival of MDS patients, the evolution in the acute myeloblastic leukemia of MDS patients and to evaluate the effect of different etiological factors on it.

Methods
Our study is a case-control study of 294 patients diagnosed with MDS from January 1999 to 2013 and followed up until 5 years after the moment of the diagnosis. Data were analyzed with IBM SPSS Statistics version 24, MedCal Software BVBA and Wizard Pro statistical software. In all the tests p < 0,05 was considered statistically important.

Results

Median incidence of MDS during the interval of time from 1999 to 2013 in the Albanian population resulted in 0,65 /100,000 inhabitants, 19-20 new cases/year. The median age at the moment of the diagnosis resulted in 61.5 ±15.08years (17 to 88-years). 65% of the patients diagnosed with MDS were at the moment of the diagnosis ≧ 60 years, with a ratio F: M ~1:1.39.  92,5% of the patients were diagnosed with primary MDS and only 7,5% were secondary MDS. According to the FAB classification, 173 patients studied retrospectively during the interval of time from 1999 to 2008 resulted: 91(53%) AREB, 28(16,2%) RA, 24(about 14%) AREB-T, 10 (5,8%) RARS, and the same result for CMML, and 9(5,2%) t- MDS. Only one patient was diagnosed with MDS-U.  During the prospective phase were diagnosed 121 pacients with MDS classified according to WHO 2008: 31(25,4%) RCMD, 29(23.8%) AREB-1, 20(16.4%)AREB-2, and 12(9,8%) t- MDS. Only two patients were diagnosed with isolated del 5q-, one patient was diagnosed with RARS and 3 (2,5%) RCMD-RS. RA were 13,9% of all the patients with MDS. OS resulted 4,6 years (95%CI 3.837 to5.441). The role of karyotype in OS (years) resulted in highly significative P < 0.001. All the MDS patients classified as higher risk (N=56/64) evolved in Acute Leukemia within 2,8 years, meanwhile, in the lower risk, MDS group resulted in 7,29 years. The presence of concomitant diseases at the moment of the diagnosis is an independent prognostic factor P =0,016 for the median survival (years) of MDS patients, but data shown no evidence of prediction in the evolution on acute myeloblastic leukemia of those patients(P=0.3346).

Conclusion
Myelodysplastic syndromes represent a heterogeneous group of malignant diseases characterized of an ineffective hematopoiesis and a tendency of evolution to acute myeloblastic leukemia. The incidence of MDS increases significantly with the age and is more frequent in males than females over 60 years old. The natural course of MDS is very different among the subgroups emphasizing the importance of the stratification and of the subclassification of those patients according to IPSS to better diagnose and treat the MDS patients.  

Keyword(s): Epidemiology, Myelodysplasia, Survival

Abstract: PB1628

Type: Publication Only

Session title: Myelodysplastic syndromes - Clinical

Background
Myelodysplastic Syndromes (MDS) compound a  heterogeneous group of malignant  hematological diseases presenting an ineffective hematopoiesis and a tendency of evolution to acute myeloblastic leukemia. The natural course of MDS is very different among the subgroups emphasizing the importance of the correct stratification / subclassification of those patients to better diagnose and treat them.  

Aims
To study the incidence of Myelodysplastic Syndromes (MDS), risk factors, concomitant diseases, genetic abnormalities at the moment of the diagnosis.Our study aims to evaluate the median survival and the overall survival of MDS patients, the evolution in the acute myeloblastic leukemia of MDS patients and to evaluate the effect of different etiological factors on it.

Methods
Our study is a case-control study of 294 patients diagnosed with MDS from January 1999 to 2013 and followed up until 5 years after the moment of the diagnosis. Data were analyzed with IBM SPSS Statistics version 24, MedCal Software BVBA and Wizard Pro statistical software. In all the tests p < 0,05 was considered statistically important.

Results

Median incidence of MDS during the interval of time from 1999 to 2013 in the Albanian population resulted in 0,65 /100,000 inhabitants, 19-20 new cases/year. The median age at the moment of the diagnosis resulted in 61.5 ±15.08years (17 to 88-years). 65% of the patients diagnosed with MDS were at the moment of the diagnosis ≧ 60 years, with a ratio F: M ~1:1.39.  92,5% of the patients were diagnosed with primary MDS and only 7,5% were secondary MDS. According to the FAB classification, 173 patients studied retrospectively during the interval of time from 1999 to 2008 resulted: 91(53%) AREB, 28(16,2%) RA, 24(about 14%) AREB-T, 10 (5,8%) RARS, and the same result for CMML, and 9(5,2%) t- MDS. Only one patient was diagnosed with MDS-U.  During the prospective phase were diagnosed 121 pacients with MDS classified according to WHO 2008: 31(25,4%) RCMD, 29(23.8%) AREB-1, 20(16.4%)AREB-2, and 12(9,8%) t- MDS. Only two patients were diagnosed with isolated del 5q-, one patient was diagnosed with RARS and 3 (2,5%) RCMD-RS. RA were 13,9% of all the patients with MDS. OS resulted 4,6 years (95%CI 3.837 to5.441). The role of karyotype in OS (years) resulted in highly significative P < 0.001. All the MDS patients classified as higher risk (N=56/64) evolved in Acute Leukemia within 2,8 years, meanwhile, in the lower risk, MDS group resulted in 7,29 years. The presence of concomitant diseases at the moment of the diagnosis is an independent prognostic factor P =0,016 for the median survival (years) of MDS patients, but data shown no evidence of prediction in the evolution on acute myeloblastic leukemia of those patients(P=0.3346).

Conclusion
Myelodysplastic syndromes represent a heterogeneous group of malignant diseases characterized of an ineffective hematopoiesis and a tendency of evolution to acute myeloblastic leukemia. The incidence of MDS increases significantly with the age and is more frequent in males than females over 60 years old. The natural course of MDS is very different among the subgroups emphasizing the importance of the stratification and of the subclassification of those patients according to IPSS to better diagnose and treat the MDS patients.  

Keyword(s): Epidemiology, Myelodysplasia, Survival

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