PMID- 8214359
OWN - NLM
STAT- MEDLINE
DCOM- 19931119
LR  - 20211203
IS  - 0192-8562 (Print)
IS  - 0192-8562 (Linking)
VI  - 15
IP  - 4
DP  - 1993 Nov
TI  - Bone marrow transplantation for Fanconi anemia. Adjustment of the dose of 
      cyclophosphamide for preconditioning.
PG  - 377-82
AB  - PURPOSE: Five patients with Fanconi anemia have been treated by bone marrow 
      transplantation. PATIENTS AND METHODS: They were conditioned with 
      cyclophosphamide (CY) (20-150 mg/kg), antilymphocyte globulin, and 
      thoracoabdominal irradiation (4-6 Gy). The dose of CY for preconditioning was 
      adjusted individually, based on the in vitro effect of CY metabolites on the 
      chromosomes of patients with Fanconi anemia. Four patients received marrow from 
      human leukocyte antigen (HLA)-identical siblings, and one received marrow from 
      his HLA phenotypically identical father. RESULTS: All patients achieved 
      engraftment, and acute graft-versus-host disease (GVHD) grade II or more was not 
      observed. Three developed chronic GVHD. All patients are surviving 2-5 years 
      after grafting, with hematological improvement. CONCLUSIONS: These results 
      indicate that the individual dose adjustment of CY used for preconditioning may 
      prevent graft failure and severe acute GVHD.
FAU - Yabe, M
AU  - Yabe M
AD  - Department of Pediatrics, Tokai University School of Medicine, Isehara, Japan.
FAU - Yabe, H
AU  - Yabe H
FAU - Matsuda, M
AU  - Matsuda M
FAU - Hinohara, T
AU  - Hinohara T
FAU - Oh, Y
AU  - Oh Y
FAU - Hattori, K
AU  - Hattori K
FAU - Ishikawa, K
AU  - Ishikawa K
FAU - Ohshima, T
AU  - Ohshima T
FAU - Yamamoto, H
AU  - Yamamoto H
FAU - Kato, S
AU  - Kato S
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Am J Pediatr Hematol Oncol
JT  - The American journal of pediatric hematology/oncology
JID - 7908071
RN  - 0 (Antilymphocyte Serum)
RN  - 8N3DW7272P (Cyclophosphamide)
SB  - IM
MH  - Anemia, Aplastic/immunology
MH  - Antilymphocyte Serum/therapeutic use
MH  - Bone Marrow Transplantation/*immunology
MH  - Child
MH  - *Chromosome Aberrations
MH  - *Chromosome Disorders
MH  - Cyclophosphamide/*therapeutic use/toxicity
MH  - Fanconi Anemia/blood/*therapy
MH  - Female
MH  - Histocompatibility Testing
MH  - Humans
MH  - Immunosuppression Therapy/*methods
MH  - Male
MH  - Tissue Donors
EDAT- 1993/11/01 00:00
MHDA- 1993/11/01 00:01
CRDT- 1993/11/01 00:00
PHST- 1993/11/01 00:00 [pubmed]
PHST- 1993/11/01 00:01 [medline]
PHST- 1993/11/01 00:00 [entrez]
PST - ppublish
SO  - Am J Pediatr Hematol Oncol. 1993 Nov;15(4):377-82.