Published in:
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
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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.