THE IMPORTANCE OF IMMUNOPHENOTYPING OF HEMOPOIETIC PRECURSORS IN THE DIAGNOSIS OF CHILDHOOD MDS
(Abstract release date: 05/19/16)
EHA Library. Lorand-Metze I. 06/09/16; 132770; E1221
Disclosure(s): I have nothing to disclose

Prof. Dr. Irene Lorand-Metze
Contributions
Contributions
Abstract
Abstract: E1221
Type: Eposter Presentation
Background
immunophenotyping of BM hemopoietic precursors has been recognized as useful ancillary technique to confirm the diagnosis of adult MDS. In children, few data about its utility are available.
Aims
to analyze retrospectively the immunophenotypic features of bone marrow (BM) cells at diagnosis of patients registered in the Brazilian Pediatric Cooperative Group of Myelodysplastic Syndromes (2012-2015).
Methods
diagnosis of the patients was based on clinical data, peripheral blood (PB) counts, BM cytology and histology and cytogenetics. Deficiency anemias, autoimmune diseases and viral infections were excluded, as well as patients with Down’s syndrome. Classification was made by the WHO Pediatric Classification. Four pediatric deficiency anemias were used as controls. Immunophenotyping was made by an 8 color platform to evaluate myelomonocytic maturation and progenitor populations according to the recommendations of the European LeukemiaNet
Results
32 patients (RCC = 6 cases; RAEB/-t = 13 cases; JMML = 13 cases) and 4 controls (deficiency anemias) were analyzed. Median age: controls = 20 months, RCC = 66 months, RAEB/-t = 68 months and JMML = 29 months. The median number of abnormalities in myeloid maturation was 1.5 (0 - 4); 3 (0 - 6) and 3 (1 - 5) respectively. The median number of abnormalities in CD34+ cells were 2 (1 - 3); 2.5 (1 - 6) and 3 (1 - 5) respectively. So, the total number of phenotypic alterations was 4 (1 - 6); 3 (2 - 11) and 6 (2 - 9) respectively. The percentage of CD34+/CD117+/CD13+ cells was 0.5% (0.1 – 2.8); 3.7 % (0.5 - 8.6) and 4.2% (0.3 - 10.1) respectively, compared to 1.1% (0.7 – 1.6) of the control cases. Aberrant cross-lineage antigen expressions in myeloid progenitors were found in 63% of the cases of JMML, in 45% of RAEB/RAEB-t but in none of the RCCs. Hematogones type I (CD34+/CD19+/CD10+) were decreased in all groups: 0.28% (0 – 2.4); 0.5% (0 – 6.4) and 0.03% (0 -0.6) respectively, compared with the controls: 2.1% (0.9 – 3.2). The same occurred in %CD34- /CD19+/CD10+ cells: 1.6% (0 – 15.5); 0.8% (0 – 6.2) and 0.02% (0. – 2.1) respectively, compared with the controls: 12.0 (3.9 – 12.9). T lymphocytes were markedly decreased only in patients with JMML. In a discriminant analysis, a model containing age of the patient, percentage of CD34+/CD117+/CD13+ cells, hematogones type I, T lymphocytes and total number of phenotypic alterations were able to classify correctly 81% of the patients.
Conclusion
in childhood MDS, alterations in myeloid precursors and CD34+ myeloid progenitors presented similar changes as in adult MDS. However, the alterations in B cell precursors and T lymphocytes were more pronounced, even in very young children. Phenotypic abnormalities in JMML were more similar to those of RAEB, regardless of the number of BM blasts counted in cytology or any feature of PB counts.
Session topic: E-poster
Type: Eposter Presentation
Background
immunophenotyping of BM hemopoietic precursors has been recognized as useful ancillary technique to confirm the diagnosis of adult MDS. In children, few data about its utility are available.
Aims
to analyze retrospectively the immunophenotypic features of bone marrow (BM) cells at diagnosis of patients registered in the Brazilian Pediatric Cooperative Group of Myelodysplastic Syndromes (2012-2015).
Methods
diagnosis of the patients was based on clinical data, peripheral blood (PB) counts, BM cytology and histology and cytogenetics. Deficiency anemias, autoimmune diseases and viral infections were excluded, as well as patients with Down’s syndrome. Classification was made by the WHO Pediatric Classification. Four pediatric deficiency anemias were used as controls. Immunophenotyping was made by an 8 color platform to evaluate myelomonocytic maturation and progenitor populations according to the recommendations of the European LeukemiaNet
Results
32 patients (RCC = 6 cases; RAEB/-t = 13 cases; JMML = 13 cases) and 4 controls (deficiency anemias) were analyzed. Median age: controls = 20 months, RCC = 66 months, RAEB/-t = 68 months and JMML = 29 months. The median number of abnormalities in myeloid maturation was 1.5 (0 - 4); 3 (0 - 6) and 3 (1 - 5) respectively. The median number of abnormalities in CD34+ cells were 2 (1 - 3); 2.5 (1 - 6) and 3 (1 - 5) respectively. So, the total number of phenotypic alterations was 4 (1 - 6); 3 (2 - 11) and 6 (2 - 9) respectively. The percentage of CD34+/CD117+/CD13+ cells was 0.5% (0.1 – 2.8); 3.7 % (0.5 - 8.6) and 4.2% (0.3 - 10.1) respectively, compared to 1.1% (0.7 – 1.6) of the control cases. Aberrant cross-lineage antigen expressions in myeloid progenitors were found in 63% of the cases of JMML, in 45% of RAEB/RAEB-t but in none of the RCCs. Hematogones type I (CD34+/CD19+/CD10+) were decreased in all groups: 0.28% (0 – 2.4); 0.5% (0 – 6.4) and 0.03% (0 -0.6) respectively, compared with the controls: 2.1% (0.9 – 3.2). The same occurred in %CD34- /CD19+/CD10+ cells: 1.6% (0 – 15.5); 0.8% (0 – 6.2) and 0.02% (0. – 2.1) respectively, compared with the controls: 12.0 (3.9 – 12.9). T lymphocytes were markedly decreased only in patients with JMML. In a discriminant analysis, a model containing age of the patient, percentage of CD34+/CD117+/CD13+ cells, hematogones type I, T lymphocytes and total number of phenotypic alterations were able to classify correctly 81% of the patients.
Conclusion
in childhood MDS, alterations in myeloid precursors and CD34+ myeloid progenitors presented similar changes as in adult MDS. However, the alterations in B cell precursors and T lymphocytes were more pronounced, even in very young children. Phenotypic abnormalities in JMML were more similar to those of RAEB, regardless of the number of BM blasts counted in cytology or any feature of PB counts.
Session topic: E-poster
Abstract: E1221
Type: Eposter Presentation
Background
immunophenotyping of BM hemopoietic precursors has been recognized as useful ancillary technique to confirm the diagnosis of adult MDS. In children, few data about its utility are available.
Aims
to analyze retrospectively the immunophenotypic features of bone marrow (BM) cells at diagnosis of patients registered in the Brazilian Pediatric Cooperative Group of Myelodysplastic Syndromes (2012-2015).
Methods
diagnosis of the patients was based on clinical data, peripheral blood (PB) counts, BM cytology and histology and cytogenetics. Deficiency anemias, autoimmune diseases and viral infections were excluded, as well as patients with Down’s syndrome. Classification was made by the WHO Pediatric Classification. Four pediatric deficiency anemias were used as controls. Immunophenotyping was made by an 8 color platform to evaluate myelomonocytic maturation and progenitor populations according to the recommendations of the European LeukemiaNet
Results
32 patients (RCC = 6 cases; RAEB/-t = 13 cases; JMML = 13 cases) and 4 controls (deficiency anemias) were analyzed. Median age: controls = 20 months, RCC = 66 months, RAEB/-t = 68 months and JMML = 29 months. The median number of abnormalities in myeloid maturation was 1.5 (0 - 4); 3 (0 - 6) and 3 (1 - 5) respectively. The median number of abnormalities in CD34+ cells were 2 (1 - 3); 2.5 (1 - 6) and 3 (1 - 5) respectively. So, the total number of phenotypic alterations was 4 (1 - 6); 3 (2 - 11) and 6 (2 - 9) respectively. The percentage of CD34+/CD117+/CD13+ cells was 0.5% (0.1 – 2.8); 3.7 % (0.5 - 8.6) and 4.2% (0.3 - 10.1) respectively, compared to 1.1% (0.7 – 1.6) of the control cases. Aberrant cross-lineage antigen expressions in myeloid progenitors were found in 63% of the cases of JMML, in 45% of RAEB/RAEB-t but in none of the RCCs. Hematogones type I (CD34+/CD19+/CD10+) were decreased in all groups: 0.28% (0 – 2.4); 0.5% (0 – 6.4) and 0.03% (0 -0.6) respectively, compared with the controls: 2.1% (0.9 – 3.2). The same occurred in %CD34- /CD19+/CD10+ cells: 1.6% (0 – 15.5); 0.8% (0 – 6.2) and 0.02% (0. – 2.1) respectively, compared with the controls: 12.0 (3.9 – 12.9). T lymphocytes were markedly decreased only in patients with JMML. In a discriminant analysis, a model containing age of the patient, percentage of CD34+/CD117+/CD13+ cells, hematogones type I, T lymphocytes and total number of phenotypic alterations were able to classify correctly 81% of the patients.
Conclusion
in childhood MDS, alterations in myeloid precursors and CD34+ myeloid progenitors presented similar changes as in adult MDS. However, the alterations in B cell precursors and T lymphocytes were more pronounced, even in very young children. Phenotypic abnormalities in JMML were more similar to those of RAEB, regardless of the number of BM blasts counted in cytology or any feature of PB counts.
Session topic: E-poster
Type: Eposter Presentation
Background
immunophenotyping of BM hemopoietic precursors has been recognized as useful ancillary technique to confirm the diagnosis of adult MDS. In children, few data about its utility are available.
Aims
to analyze retrospectively the immunophenotypic features of bone marrow (BM) cells at diagnosis of patients registered in the Brazilian Pediatric Cooperative Group of Myelodysplastic Syndromes (2012-2015).
Methods
diagnosis of the patients was based on clinical data, peripheral blood (PB) counts, BM cytology and histology and cytogenetics. Deficiency anemias, autoimmune diseases and viral infections were excluded, as well as patients with Down’s syndrome. Classification was made by the WHO Pediatric Classification. Four pediatric deficiency anemias were used as controls. Immunophenotyping was made by an 8 color platform to evaluate myelomonocytic maturation and progenitor populations according to the recommendations of the European LeukemiaNet
Results
32 patients (RCC = 6 cases; RAEB/-t = 13 cases; JMML = 13 cases) and 4 controls (deficiency anemias) were analyzed. Median age: controls = 20 months, RCC = 66 months, RAEB/-t = 68 months and JMML = 29 months. The median number of abnormalities in myeloid maturation was 1.5 (0 - 4); 3 (0 - 6) and 3 (1 - 5) respectively. The median number of abnormalities in CD34+ cells were 2 (1 - 3); 2.5 (1 - 6) and 3 (1 - 5) respectively. So, the total number of phenotypic alterations was 4 (1 - 6); 3 (2 - 11) and 6 (2 - 9) respectively. The percentage of CD34+/CD117+/CD13+ cells was 0.5% (0.1 – 2.8); 3.7 % (0.5 - 8.6) and 4.2% (0.3 - 10.1) respectively, compared to 1.1% (0.7 – 1.6) of the control cases. Aberrant cross-lineage antigen expressions in myeloid progenitors were found in 63% of the cases of JMML, in 45% of RAEB/RAEB-t but in none of the RCCs. Hematogones type I (CD34+/CD19+/CD10+) were decreased in all groups: 0.28% (0 – 2.4); 0.5% (0 – 6.4) and 0.03% (0 -0.6) respectively, compared with the controls: 2.1% (0.9 – 3.2). The same occurred in %CD34- /CD19+/CD10+ cells: 1.6% (0 – 15.5); 0.8% (0 – 6.2) and 0.02% (0. – 2.1) respectively, compared with the controls: 12.0 (3.9 – 12.9). T lymphocytes were markedly decreased only in patients with JMML. In a discriminant analysis, a model containing age of the patient, percentage of CD34+/CD117+/CD13+ cells, hematogones type I, T lymphocytes and total number of phenotypic alterations were able to classify correctly 81% of the patients.
Conclusion
in childhood MDS, alterations in myeloid precursors and CD34+ myeloid progenitors presented similar changes as in adult MDS. However, the alterations in B cell precursors and T lymphocytes were more pronounced, even in very young children. Phenotypic abnormalities in JMML were more similar to those of RAEB, regardless of the number of BM blasts counted in cytology or any feature of PB counts.
Session topic: E-poster
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