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СLINICAL AND HEMATOLOGICAL FEATURES OF PRIMARY MYELODYSPLASTIC SYNDROMES IN ADULTS AND CHILDREN
Author(s): ,
Natalia Klimkovich
Affiliations:
Department of Pediatric Oncology and Hematology,Byelorussian Medical Academy of Post-Graduate Education,Minsk,Belarus
Olga Krasko
Affiliations:
Laboratory of Bioinformatics,United Institute of Informatics Problems of the National Academy of Sciences,Minsk,Belarus
(Abstract release date: 05/19/16) EHA Library. Klimkovich N. 06/09/16; 132775; E1226
Mrs. Natallia Klimkovich
Mrs. Natallia Klimkovich
Contributions
Abstract
Abstract: E1226

Type: Eposter Presentation

Background
Myelodysplastic syndromes (MDS) are included into a heterogeneous group of clonal blood diseases characterized by peripheral cytopenias, dysplastic features of hematopoietic precursors, progressive deterioration and a high risk of transformation into leukemia. MDS occur in several versions that differ in frequency of appearance, the duration of the course and the probability of transformation into acute leukemia. There are differences in the structure of MDS variants in patients of different age. This is the basis for the discussion of clinical and hematological characteristics of primary MDS and systematization of the results.

Aims
Analysis of the clinical and laboratory characteristics of patients with primary MDS, depending on age.

Methods
The study included 162 (144 adults and 18 children) with primary MDS patients aged from 1.5 to 60 years (median age 49 years, adults, children - 8 years). The material of the study were clinical and medical history, peripheral blood (PC) and bone marrow (BM). Clinical criteria for inclusion of patients in the study: a verified diagnosis of MDS, setting variant of the disease in accordance with the criteria of the WHO classification of myeloid neoplasms (2008), written informed consent for inclusion in the study. The calculations are performed in the R version 3.1.3 statistical package.

Results
Primary MDS have a features of clinical - hematological manifestations in the age groups under 18 years, 18-39 years and 40-60 years, with the result that appears different variants of structure (p=0.028) and by IPSS risk categories (p=0.001). IPSS demonstrates a high prognostic significance in adult patients (p<0.001), in contrast to pediatric MDS (p=0.110). Most cases of MDS in children have been the normocytic anemia (MCV 86,5 (73 ... 103) fl). While MDS for adults, irrespective of age, a feature of dysplasia is macrocytosis (MCV 101,5 (83 ... 123) fl), p<0.001. In children occur the hypocellularity BM in 22.2%. Adult patients have this characteristic in 2.8% of the age group of 18-39 years and there is no group in the 40-60 years of age (p <0.001). Erythroid hyperplasia BM and availability of microforms megakaryocytes BM are frequent signs of MDS in adults (72.2% and 94.4% in the age group 18-39 years, 62% and 92.6% in the age group 40-60 years, respectively). In children occur this feature only 22.2% and 66.7% of cases (p <0,001 and p= 0.002, respectively). Many young granulocytes in BM biopsy (p = 0.022) and BM stroma fibrosis (p = 0.036) more common in MDS in the age group 40-60 years compared to patients under 18 years and 18-39 years. A normal karyotype was detected in 71.8% of adults and 77.8% of children with MDS. In adult patients with MDS with increasing age decreases the frequency of normal karyotype with an increase in the number of unbalanced and complex aberrations. Children with MDS were observed with the same frequency the isolated and complex cytogenetic damage.

Conclusion
Patients with MDS of different age groups (children 18-40 years and 40-60 years) have variable manifestations of MDS, which is determined by the structure of variants, frequency of cytogenetic and dysplastic abnormalities BM cells, the intensity of the progression. Childhood MDS is a separate group of disease with features of clinical and hematological manifestations different from adults, which makes the feasibility of its version in the classification.

Session topic: E-poster

Keyword(s): Age, Clinical data, MDS
Abstract: E1226

Type: Eposter Presentation

Background
Myelodysplastic syndromes (MDS) are included into a heterogeneous group of clonal blood diseases characterized by peripheral cytopenias, dysplastic features of hematopoietic precursors, progressive deterioration and a high risk of transformation into leukemia. MDS occur in several versions that differ in frequency of appearance, the duration of the course and the probability of transformation into acute leukemia. There are differences in the structure of MDS variants in patients of different age. This is the basis for the discussion of clinical and hematological characteristics of primary MDS and systematization of the results.

Aims
Analysis of the clinical and laboratory characteristics of patients with primary MDS, depending on age.

Methods
The study included 162 (144 adults and 18 children) with primary MDS patients aged from 1.5 to 60 years (median age 49 years, adults, children - 8 years). The material of the study were clinical and medical history, peripheral blood (PC) and bone marrow (BM). Clinical criteria for inclusion of patients in the study: a verified diagnosis of MDS, setting variant of the disease in accordance with the criteria of the WHO classification of myeloid neoplasms (2008), written informed consent for inclusion in the study. The calculations are performed in the R version 3.1.3 statistical package.

Results
Primary MDS have a features of clinical - hematological manifestations in the age groups under 18 years, 18-39 years and 40-60 years, with the result that appears different variants of structure (p=0.028) and by IPSS risk categories (p=0.001). IPSS demonstrates a high prognostic significance in adult patients (p<0.001), in contrast to pediatric MDS (p=0.110). Most cases of MDS in children have been the normocytic anemia (MCV 86,5 (73 ... 103) fl). While MDS for adults, irrespective of age, a feature of dysplasia is macrocytosis (MCV 101,5 (83 ... 123) fl), p<0.001. In children occur the hypocellularity BM in 22.2%. Adult patients have this characteristic in 2.8% of the age group of 18-39 years and there is no group in the 40-60 years of age (p <0.001). Erythroid hyperplasia BM and availability of microforms megakaryocytes BM are frequent signs of MDS in adults (72.2% and 94.4% in the age group 18-39 years, 62% and 92.6% in the age group 40-60 years, respectively). In children occur this feature only 22.2% and 66.7% of cases (p <0,001 and p= 0.002, respectively). Many young granulocytes in BM biopsy (p = 0.022) and BM stroma fibrosis (p = 0.036) more common in MDS in the age group 40-60 years compared to patients under 18 years and 18-39 years. A normal karyotype was detected in 71.8% of adults and 77.8% of children with MDS. In adult patients with MDS with increasing age decreases the frequency of normal karyotype with an increase in the number of unbalanced and complex aberrations. Children with MDS were observed with the same frequency the isolated and complex cytogenetic damage.

Conclusion
Patients with MDS of different age groups (children 18-40 years and 40-60 years) have variable manifestations of MDS, which is determined by the structure of variants, frequency of cytogenetic and dysplastic abnormalities BM cells, the intensity of the progression. Childhood MDS is a separate group of disease with features of clinical and hematological manifestations different from adults, which makes the feasibility of its version in the classification.

Session topic: E-poster

Keyword(s): Age, Clinical data, MDS

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