title: Successful bone marrow plus cord blood stem cell transplantation in a girl who developed myelodysplastic syndrome from hepatitis-associated aplastic anemia treated with long-term immunosuppressants and growth factors.
abstract: A 9-year-old girl who had hepatitis-associated aplastic anemia was treated intermittently with methylprednisolone pulse therapy and growth factors (granulocyte-colony stimulating factor (G-CSF), recombinant human erythropoietin (rhEpo) and cyclosporin A (CyA) for over two years. At this time, there was hematological improvement, but chromosome analysis revealed monosomy 7. After six months, there was progression to myelodysplastic syndrome (MDS) (stage in refractory anemia of excess blasts (RAEB)) with monosomy 7, monosomy 6, marker chromosome and with hematological deterioration. She received bone marrow (1.57 x 10(5) cells kg(-1) (patient body weight)) plus cord blood cell (0.3 x 10(7) cells kg(-1) (patient body weight)) transplantation from her brother, 2 years and 7 months after the diagnosis of hepatitis-associated aplastic anemia. Engraftment was achieved after two weeks, and acute graft-versus-host disease occurred in a mild form after four weeks. Hematological remission has been continuous for 20 months after bone marrow transplantation. Transformation of hepatitis-associated aplastic anemia to MDS with the monosomy 7, monosomy 6 and marker chromosome in this patient was considered to have been related to the administration of high doses of immunosuppressive drugs plus growth factors.
authors.affiliation: Department of Pediatrics, Saitama Medical School, 38 Morohongo Iruma-gun, Saitama, Japan 350-0495. ashibuya@saitama-med.ac.jp
authors.forename: Atsushi
authors.initials: A
authors.lastname: Shibuya
authors.forename: Saori
authors.initials: S
authors.lastname: Ishii
authors.forename: Kaoru
authors.initials: K
authors.lastname: Obinata
mesh_terms: D000741:Anemia, Aplastic / Q000150:complications* / Q000188:drug therapy; D016026:Bone Marrow Transplantation; D002648:Child; D002896:Chromosomes, Human, Pair 6; D002897:Chromosomes, Human, Pair 7; D036101:Cord Blood Stem Cell Transplantation; D004921:Erythropoietin / Q000009:adverse effects / Q000627:therapeutic use; D005260:Female; D016179:Granulocyte Colony-Stimulating Factor / Q000009:adverse effects / Q000627:therapeutic use; D006133:Growth Substances / Q000009:adverse effects* / Q000627:therapeutic use; D006505:Hepatitis / Q000150:complications; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects* / Q000627:therapeutic use; D009006:Monosomy; D009190:Myelodysplastic Syndromes / Q000139:chemically induced* / Q000235:genetics / Q000628:therapy*; D011994:Recombinant Proteins; D014184:Transplantation, Homologous; D016896:Treatment Outcome
chemical_list: D006133:Growth Substances; D007166:Immunosuppressive Agents; D011994:Recombinant Proteins; D004921:Erythropoietin; D016179:Granulocyte Colony-Stimulating Factor