Abstract
Bone marrow transplantation (BMT) was done three times for a 6-year-old girl with myelodysplastic syndrome (MDS). She showed progressive bicytopenia and constitutional abnormalities including short stature and skin pigmentation. We initially diagnosed the patient as having Fanconi's anemia, although the chromosome fragility was not demonstrated by the mitomycin C stress test. The patient received conditioning regimens which consisted of 3 Gy of total body irradiation followed by decreased dose of cyclophosphamide (CY, 10 mg/kg for 4 days) and anti-lymphocyte globulin (ALG, 30 mg/kg for 5 days) and marrow cells from HLA-identical 8-year-old brother were given. However, the graft was rejected. Reviewing the previous bone marrow smears disclosed the abnormalities in the three major cell lines : erythroblasts, granulocytes, megakaryocytes. Therefore, we considered her to have MDS. The second BMT was done with busulfan (4 mg/kg for 4 days) followed by CY (60 mg/kg for 2 days); however, no evidence of engraftment occurred until day 25. Therefore, she received CY (50 mg/kg for 4 days), ALG (40 mg/kg for 4 days) and the third BMT with successful engraftment which was confirmed by the chromosomal karyotype on day 19. She showed remarkable hematological recovery and bone marrow examination on day 54 showed complete disappearance of morphological abnormalities of all cell lineages seen prior to BMT. She is well, with full performance state.
