ABSTRACT
We report an 11-year old female with myelodysplastic (refractory anemia with excess of blasts) presentation of Fanconi anemia. After failure of initial chemotherapy with low doses of 6-mercaptopurine and prednisolone she underwent allogeneic bone marrow transplantation (BMT) from her HLA-matched sibling. Busulfan 8 mg/kg and cyclophosphamide 40 mg/kg were used as conditioning. The post-transplant course was uneventful with fast trilineage engraftment and mild cutaneous acute GVHD. She is alive 17 months after BMT with full hematological reconstitution without evidence of MDS.
Subject(s)
Alkylating Agents/administration & dosage , Anemia, Refractory, with Excess of Blasts/therapy , Bone Marrow Transplantation , Busulfan/administration & dosage , Cyclophosphamide/administration & dosage , Fanconi Anemia/therapy , Transplantation Conditioning , Anemia, Refractory, with Excess of Blasts/drug therapy , Anemia, Refractory, with Excess of Blasts/pathology , Child , Combined Modality Therapy , Drug Therapy, Combination , Fanconi Anemia/drug therapy , Fanconi Anemia/pathology , Female , Humans , Transplantation, HomologousABSTRACT
Investigation of the distribution of HLA antigens in children with acute lymphoblastic leukemia demonstrated a significant increase of the B17 antigen frequency and a decrease of the frequency of DR7 antigen. A high index of the relative risk (RR-3.207) was also revealed for antigen B40 carriers. The typing of the children suffering from acute lymphoblastic leukemia should be carried out, as shown by the investigations, based on the immunocytological variant of acute lymphoblastic leukemia.
