PMID- 9021815 OWN - NLM STAT- MEDLINE DCOM- 19970415 LR - 20191024 IS - 0888-0018 (Print) IS - 0888-0018 (Linking) VI - 14 IP - 1 DP - 1997 Jan-Feb TI - Bone marrow transplantation in patients with Fanconi anemia: experience with cyclophosphamide and total body irradiation conditioning regimen. PG - 67-72 AB - Eleven patients with Fanconi anemia (FA) underwent bone marrow transplantation (BMT) between March 1985 and May 1990 in a single institution. Ten patients received bone marrow from healthy full human leukocyte antigen (HLA) matched siblings and one patient from her father (one antigen mismatch). Ten patients were conditioned with cyclophosphamide (Cy) at a dose of 5 mg/kg per day for 4 days followed by total body irradiation (TBI) for a total of 600 cGy over 3 days. Six of the 11 patients are alive and have normal reconstitution of their bone marrow. Median follow-up was 72 months (range 42-84). Three of the 10 patients who received Cy and TBI (two HLA compatible, one antigen mismatch) had graft failure. Five patients developed at least grade III acute graft-versus-host disease (GVHD). The rates of graft failure and GVHD are, however, still significantly high. Modification of the conditioning regimen and GVHD prophylaxis is needed to improve the outcome. FAU - Solh, H AU - Solh H AD - Department of Oncology, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia. FAU - Rao, K AU - Rao K FAU - Martins da Cunha, A M AU - Martins da Cunha AM FAU - Padmos, A AU - Padmos A FAU - Sackey, K AU - Sackey K FAU - Ernst, P AU - Ernst P FAU - Spence, D AU - Spence D FAU - Clink, H AU - Clink H LA - eng PT - Journal Article PL - England TA - Pediatr Hematol Oncol JT - Pediatric hematology and oncology JID - 8700164 RN - 0 (Immunosuppressive Agents) RN - 8N3DW7272P (Cyclophosphamide) SB - IM MH - Adolescent MH - *Bone Marrow Transplantation MH - Child MH - Child, Preschool MH - Cyclophosphamide/*therapeutic use MH - Fanconi Anemia/*therapy MH - Female MH - Humans MH - Immunosuppressive Agents/*therapeutic use MH - Male MH - Retrospective Studies MH - Transplantation Conditioning/*methods MH - Treatment Outcome MH - *Whole-Body Irradiation EDAT- 1997/01/01 00:00 MHDA- 1997/01/01 00:01 CRDT- 1997/01/01 00:00 PHST- 1997/01/01 00:00 [pubmed] PHST- 1997/01/01 00:01 [medline] PHST- 1997/01/01 00:00 [entrez] AID - 10.3109/08880019709030886 [doi] PST - ppublish SO - Pediatr Hematol Oncol. 1997 Jan-Feb;14(1):67-72. doi: 10.3109/08880019709030886.