Skip to main content
Top
Published in: International Journal of Hematology 5/2008

01-12-2008 | Original Article

Clinicopathological characteristics of erythroblast-rich RAEB and AML M6a in children

Authors: Yuko Honda, Atsushi Manabe, Masahiro Tsuchida, Yuji Zaike, Atsuko Masunaga, Masami Inoue, Ryoji Kobayashi, Yoshitoshi Ohtsuka, Akira Kikuchi, Tatsutoshi Nakahata, From the MDS Committee, the Japanese Society of Pediatric Hematology

Published in: International Journal of Hematology | Issue 5/2008

Login to get access

Abstract

The distinction between RAEB, RAEB-T and AML M6a is difficult when erythroblasts in the bone marrow (BM) exceed 50%. We analyzed 19 children (2 RAEB, 13 RAEB-T and 4 AML M6a) enrolled in a prospective pathological central review in Japan and divided them into two groups according to the myeloblasts percentage among non-erythroid cells in BM: group A (n = 8), 5–19% myeloblasts; group B (n = 11), 20% or more myeloblasts. Their characteristics were very similar except for the number of myeloblasts. The median WBC was in the range of 1.0–5.0 × 109 L−1, the median Hb was around 7.5 g/dL, the median MCV was greater than 90 fL and both group had Auer rods at 60–65%. Severe multilineage dysplasia was observed in most of the patients in two groups. Six with group A and seven with group B treated with AML type chemotherapy achieved complete remission. Five with group A and seven with group B undergoing SCT are alive at a median of 3 years after diagnosis. Erythroblast-rich RAEB and AML M6a in children have similar characteristics and may belong to a single disease entity.
Literature
1.
go back to reference Bennett JM, Catovsky D, Daniei MT, Flandrin G, Galton DAG, Garlnick HR, et al. Proposals for the classification of the myelodysplastic syndromes. Br J Haematol. 1982;51:189–99.CrossRefPubMed Bennett JM, Catovsky D, Daniei MT, Flandrin G, Galton DAG, Garlnick HR, et al. Proposals for the classification of the myelodysplastic syndromes. Br J Haematol. 1982;51:189–99.CrossRefPubMed
2.
go back to reference Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman J. The World Health Organization Classification of neoplastic diseases of the hematopoietic and lymphoid tissues: report of the clinical advisory committee meeting, Airlie House, Virginia, November, 1997. Ann Oncol. 1999;10:1419–32. doi:10.1023/A:1008375931236.CrossRefPubMed Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman J. The World Health Organization Classification of neoplastic diseases of the hematopoietic and lymphoid tissues: report of the clinical advisory committee meeting, Airlie House, Virginia, November, 1997. Ann Oncol. 1999;10:1419–32. doi:10.​1023/​A:​1008375931236.CrossRefPubMed
3.
4.
go back to reference Rosati S, Anastasi J, Vardiman J. Recurring diagnostic problems in the pathology of the myelodysplastic syndromes. Semin Hematol. 1996;33:111–26.PubMed Rosati S, Anastasi J, Vardiman J. Recurring diagnostic problems in the pathology of the myelodysplastic syndromes. Semin Hematol. 1996;33:111–26.PubMed
6.
go back to reference Sasaki H, Manabe A, Kojima S, Tsuchida M, Hayashi Y, Ikuta K, et al. Myelodysplastic syndrome in childhood: a retrospective study of 189 patients in Japan. Leukemia. 2001;15:1713–20.CrossRefPubMed Sasaki H, Manabe A, Kojima S, Tsuchida M, Hayashi Y, Ikuta K, et al. Myelodysplastic syndrome in childhood: a retrospective study of 189 patients in Japan. Leukemia. 2001;15:1713–20.CrossRefPubMed
7.
go back to reference Manabe A. Recent progress in childhood myelodysplastic syndrome. Japan J Pediatr Hematol. 2007;21:141–51. (in Japanese). Manabe A. Recent progress in childhood myelodysplastic syndrome. Japan J Pediatr Hematol. 2007;21:141–51. (in Japanese).
9.
go back to reference Cheson BD, Greenberg PL, Bennett JM, Lowenberg B, Wijermans PW, Nimer SD, et al. Clinical application and proposal for modification of the International Working Group (IWG) response criteria in myelodysplasia. Blood. 2006;108:419–25. doi:10.1182/blood-2005-10-4149.CrossRefPubMed Cheson BD, Greenberg PL, Bennett JM, Lowenberg B, Wijermans PW, Nimer SD, et al. Clinical application and proposal for modification of the International Working Group (IWG) response criteria in myelodysplasia. Blood. 2006;108:419–25. doi:10.​1182/​blood-2005-10-4149.CrossRefPubMed
10.
go back to reference Strupp C, Gattermann N, Giagounidis A, Giagounidis A, Aul C, Hildebrandt B, et al. Refractory anemia with excess of blasts in transformation: analysis of reclassification according to the WHO proposals. Leuk Res. 2003;27:397–404. doi:10.1016/S0145-2126(02)00220-5.CrossRefPubMed Strupp C, Gattermann N, Giagounidis A, Giagounidis A, Aul C, Hildebrandt B, et al. Refractory anemia with excess of blasts in transformation: analysis of reclassification according to the WHO proposals. Leuk Res. 2003;27:397–404. doi:10.​1016/​S0145-2126(02)00220-5.CrossRefPubMed
12.
go back to reference Webb DKH, Harrison G, Stevens RF, Gibson BG, Hann I, Wheatley K. Relationships between age at diagnosis, clinical features, and outcome of therapy in children treated in the Medical Research Council AML 10 and 12 trials for acute myeloid leukemia. Blood. 2001;98:1714–20. doi:10.1182/blood.V98.6.1714.CrossRefPubMed Webb DKH, Harrison G, Stevens RF, Gibson BG, Hann I, Wheatley K. Relationships between age at diagnosis, clinical features, and outcome of therapy in children treated in the Medical Research Council AML 10 and 12 trials for acute myeloid leukemia. Blood. 2001;98:1714–20. doi:10.​1182/​blood.​V98.​6.​1714.CrossRefPubMed
13.
go back to reference Barnard DR, Alonzo TA, Gerbing RB, Lange B, Woods WG. Comparison of childhood myelodysplastic syndrome, AML FAB M6 or M7, CCG 2891: report from the children’s oncology group. Pediatr Blood Cancer. 2007;49:17–22. doi:10.1002/pbc.20951.CrossRefPubMed Barnard DR, Alonzo TA, Gerbing RB, Lange B, Woods WG. Comparison of childhood myelodysplastic syndrome, AML FAB M6 or M7, CCG 2891: report from the children’s oncology group. Pediatr Blood Cancer. 2007;49:17–22. doi:10.​1002/​pbc.​20951.CrossRefPubMed
14.
go back to reference Niemeyer C, Duffner U, Bender-Gotze C, et al. AML-intensive chemotherapy prior to stem cell transplantation (SCT) does not improve survival in children and adolescents with primary myelodysplastic sundrome (MDS). Blood. 2000;96(Suppl 1):521a. abstract. Niemeyer C, Duffner U, Bender-Gotze C, et al. AML-intensive chemotherapy prior to stem cell transplantation (SCT) does not improve survival in children and adolescents with primary myelodysplastic sundrome (MDS). Blood. 2000;96(Suppl 1):521a. abstract.
15.
17.
go back to reference Park S, Picard F, Azgui Z, Viguie F, Merlat A, Guesnu M, et al. Erythroleukemia: a comparison between the previous FAB approach and the WHO classification. Leuk Res. 2002;26:423–9.CrossRefPubMed Park S, Picard F, Azgui Z, Viguie F, Merlat A, Guesnu M, et al. Erythroleukemia: a comparison between the previous FAB approach and the WHO classification. Leuk Res. 2002;26:423–9.CrossRefPubMed
Metadata
Title
Clinicopathological characteristics of erythroblast-rich RAEB and AML M6a in children
Authors
Yuko Honda
Atsushi Manabe
Masahiro Tsuchida
Yuji Zaike
Atsuko Masunaga
Masami Inoue
Ryoji Kobayashi
Yoshitoshi Ohtsuka
Akira Kikuchi
Tatsutoshi Nakahata
From the MDS Committee, the Japanese Society of Pediatric Hematology
Publication date
01-12-2008
Publisher
Springer Japan
Published in
International Journal of Hematology / Issue 5/2008
Print ISSN: 0925-5710
Electronic ISSN: 1865-3774
DOI
https://doi.org/10.1007/s12185-008-0183-3

Other articles of this Issue 5/2008

International Journal of Hematology 5/2008 Go to the issue
Webinar | 19-02-2024 | 17:30 (CET)

Keynote webinar | Spotlight on antibody–drug conjugates in cancer

Antibody–drug conjugates (ADCs) are novel agents that have shown promise across multiple tumor types. Explore the current landscape of ADCs in breast and lung cancer with our experts, and gain insights into the mechanism of action, key clinical trials data, existing challenges, and future directions.

Dr. Véronique Diéras
Prof. Fabrice Barlesi
Developed by: Springer Medicine