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

• Published in: International journal of hematology Vol. 88; no. 5; pp. 524 - 529
• Main Authors: Honda, Yuko, Manabe, Atsushi, Tsuchida, Masahiro, Zaike, Yuji, Masunaga, Atsuko, Inoue, Masami, Kobayashi, Ryoji, Ohtsuka, Yoshitoshi, Kikuchi, Akira, Nakahata, Tatsutoshi
• Format: Journal Article
• Language: English
• Published: Tokyo Springer Japan 01.12.2008; Springer; Springer Nature B.V
• Subjects: Adolescent; Anemia, Refractory, with Excess of Blasts - blood; Anemia, Refractory, with Excess of Blasts - mortality; Anemia, Refractory, with Excess of Blasts - pathology; Anemia, Refractory, with Excess of Blasts - therapy; Biological and medical sciences; Child; Child, Preschool; Erythroblasts - metabolism; Erythroblasts - pathology; Female; Granulocyte Precursor Cells - metabolism; Granulocyte Precursor Cells - pathology; Hematologic and hematopoietic diseases; Hematology; Humans; Infant; Japan; Leukemia, Myeloid, Acute - blood; Leukemia, Myeloid, Acute - mortality; Leukemia, Myeloid, Acute - pathology; Leukemia, Myeloid, Acute - therapy; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Leukocyte Count; Male; Medical sciences; Medicine; Medicine & Public Health; Oncology; Original Article; Prospective Studies; Remission Induction; Stem Cell Transplantation; Time Factors; Transplantation, Homologous; Japan; AML M6a; RAEB; Children; Auer rods; Myelodysplastic syndrome; Erythroblast; Human; Acute myelogenous leukemia; Hematology; Malignant hemopathy; Refractory anemia with excess blasts; Auer body; Myelodysplastic syndrome . Children; Child; Cancer
• ISSN: 0925-5710; 1865-3774
• DOI: 10.1007/s12185-008-0183-3

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 × 10 9  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.