Residual hemopoiesis and myelodysplasia development in aplastic anemia children. -Epidemiologic and clinical observation

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Although both of an aplastic anemia (AA) and a myelodysplastic syndrome(MDS)-refractory anemia(RA) are bone marrow failure syndrome, disease-overlapping between these disease has been discussed. Aim of this study is clarified that etiology and clinical characteristics of MDS-developed AA patient and residual hemopoiesis in these bone marrow failure syndrome.According to database that has been established by Japanese Society of Pediatric Hematology, a prognosis of MDS developed AA patient depends on chromosome abnormalities. Trisomy 8 is good, however monosomy 7 is poor prognostic marker. Incidence of MDS development in RA patients was low. Clinical and hematological response for immunosuppressive therapy (IST) was suspected as high as 50-60% in both AA and MDS-RA. MCV was also high in both type of bone marrow failure because MCV reflects ineffective erythropoiesis.For the purpose of evaluation for residual hemopoiesis, multidimensional flow cytometry analysis (MDF) was introduced in a small-scale study. As a tentative result, MDF technique has a possibility to detect exhaustion of immature myeloid cells at an AA diagnosis and was able to follow hemopoiesis recovery after IST, and also detect aberrant Fells on abnormal maturation pathway.