Showing papers by "Eliane Gluckman published in 1980"
TL;DR: Five patients with Fanconi anaemia treated by bone marrow transplantation from HLA identical donors only one patient survived for more than 3 years, indicating a special sensitivity of FA cells to alkylating agents and the need to modify the conditioning regimen in FA patients.
Abstract: Summary Five patients with Fanconi anaemia have been treated by bone marrow transplantation from HLA identical donors. Only one patient survived for more than 3 years. She is now perfectly healthy with complete haematological reconstitution with chimaerism and disparition of chromosomal abnormalities. In contrast, four patients died of acute severe GVHD soon after grafting. In addition, all had signs of severe cyclophosphamide toxicity. This evolution could be explained by a special sensitivity of FA cells to alkylating agents and may indicate the need to modify the conditioning regimen in FA patients.
143 citations
TL;DR: The high susceptibility of Fanconi anaemia patients chromosomes was observed when low concentrations of sera of a cyclophosphamide‐treated patient was added to PHA‐stimulated lymphocyte cultures, and the high sensitivity of FA patients to cycloph phosphate when used as a conditioning drug for bone marrow graft is discussed.
Abstract: Summary The effect of cyclophosphamide metabolites was studied on chromosomes of Fanconi anaemia patients, parents and controls. A high susceptibility of FA patients chromosomes was observed when low concentrations of sera of a cyclophosphamide-treated patient was added to PHA-stimulated lymphocyte cultures. No effect was observed with comparable concentrations on cells from FA patients or controls. The high susceptibility of FA cells is discussed in relation to the high sensitivity of FA patients to cyclophosphamide when used as a conditioning drug for bone marrow graft.
101 citations
41 citations
38 citations
TL;DR: Lymphocyte chromosome studies before and after allogenic graft of a Fanconi's anemia patient showed that chromosome breakage disappeared following grafting, demonstrating that the defect responsible for chromosomes breakage lies in the FA cell itself.
10 citations
5 citations
TL;DR: No statistically significant correlation could be found between the presence of auto- or allo- LTs, either before or after BMT, and the clinical evolution, but different patterns of antibody production were noted in relation to graft outcome.
Abstract: Forty-seven recipients with aplastic anemia who received a bone marrow transplant (BMT) from an HLA-identical sibling have been studied. Sera were analyzed after platelet absorption, and they were tested on target cells from a panel of unrelated donors, from the recipient, and his family. Sixty-two percent of the patients displayed lymphocytotoxins (LTs) that were directed to non-HLA antigens, and that reacted with B and/or T lymphocyte subpopulations. These LTs were temperature sensitive with maximum activity detectable at 20 or 15 C, exceptionally at 37 C. In 16 patients, LTs reacted with the recipients' as with other allogeneic cells and they were referred to as autoantibodies, while in 13 cases they were only alloantibodies. When auto-LTs reacted against both B and T lymphocytes, the percentage of killing on the B cell panel was usually larger than that on the T cell panel, which suggests that the membrane determinants involved are present at a higher density on B cells and at a lower density on T cells or that, at least in some instances, LTs do not recognize the same specificities on B and T cells. However absorption on either T or B lymphocytes completely removed reactivity to both of these cell populations. No statistically significant correlation could be found between the presence of auto- or allo- LTs, either before or after BMT, and the clinical evolution. However, different patterns of antibody production were noted in relation to graft outcome:LTs appeared during the 2nd or 3rd month and were transient in the patients with graft take, they occurred earlier on and persisted in case of graft-versus-host disease (GVHD), and they appeared during rejection and waned once the rejection process was completed.
3 citations
TL;DR: In six patients undergoing allogeneic bone marrow transplantation granulocyte colony and cluster forming units were detectable in the blood up to 24 hours after intravenous marrow infusion, and no correlation of the blood CFU-C decay curve with the subsequent fate of the graft was observed.
Abstract: Summary
In six patients undergoing allogeneic bone marrow transplantation granulocyte colony and cluster forming units were detectable in the blood up to 24 hours after intravenous marrow infusion. The mean surviving fraction 30 minutes after the end of the infusion was 0.3 for colonies and 0.42 for total aggregates. CFU-C declined logarithmically with time, but a small secondary rise in blood CFU-C occurred 2-6 hours after the end of the marrow infusion. Three patients were plasma-exchanged immediately before the marrow graft for major donor-recipient ABO incompatability. The proportion of total groups remaining in the blood at 30 minutes was significantly higher in the plasma-pheresed patients (P = < 0.05), and the secondary rise in CFU-C was less marked, but no correlation of the blood CFU-C decay curve with the subsequent fate of the graft was observed.