Journal ArticleDOI
Hematopoietic reconstitution in a patient with fanconi's anemia by means of umbilical-cord blood from an hla-identical sibling
Eliane Gluckman,Hal E. Broxmeyer,Arleen D. Auerbach,Henry S. Friedman,Gordon W. Douglas,Agnès Devergie,Helene Esperou,Dominique Thierry,Gérard Socié,Pierre Lehn,Scott Cooper,Denis English,Joanne Kurtzberg,Judith Bard,Edward A. Boyse +14 more
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TLDR
It is necessary to select patients suitable for vaginal or laparoscopic mesh placement for Fanconi's anemia preoperatively on the basis of prior history and once they provide informed consent for surgery.Abstract:
The clinical manifestations of Fanconi’s anemia, an autosomal recessive disorder, include progressive pancytopenia, a predisposition to neoplasia, and nonhematopoietic developmental anomalies [1-3]. Hypersensitivity to the clastogenic effect of DNA-cross-linking agents such as diepoxybutane acts as a diagnostic indicator of the genotype of Fanconi’s anemia, both prenatally and postnatally [3-6]. Prenatal HLA typing has made it possible to ascertain whether a fetus is HLA-identical to an affected sibling [7]. We report here on hematopoietic reconstitution in a boy with severe Fanconi’s anemia who received cryo-preserved umbilical-cord blood from a sister shown by prenatal testing to be unaffected by the disorder, to have a normal karyotype, and to be HLA-identical to the patient. We used a pretransplantation conditioning procedure developed specifically for the treatment of such patients [8]; this technique makes use of the hypersensitivity of the abnormal cells to alkylating agents that cross-link DNA [9,10] and to irradiation [11] In this case, the availability of cord blood obviated the need for obtaining bone marrow from the infant sibling. This use of cord blood followed the suggestion of one of us that blood retrieved from umbilical cord at delivery, usually discarded, might restore hematopoiesis – a proposal supported by preparatory studies by some of us [12] and consistent with reports on the presence of hematopoietic stem and multipotential (CFU-GEMM), erythroid (BFU-E), and granulocyte-macrophage (CFU-GM) progenitor cells in human umbilical-cord blood (see the references cited by Broxmeyer et al. [12]).read more
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Allogeneic cord blood transplantation in children with hematological malignancies: a long-term follow-up single-center study.
Miguel Angel Diaz,Marta González-Vicent,Manuel Ramírez,Julián Sevilla,Alvaro Lassaletta,Antonio Pérez,Luis Madero +6 more
TL;DR: The results from a long-term follow-up study suggest that cord blood transplantation should be performed in the early phase of disease whenever possible and the cord blood unit for transplantation in pediatric patients with hematological malignancies should be chosen based on cell dose, especially a CD34+ cell dose.
Journal ArticleDOI
Attached segment has higher CD34+ cells and CFU-GM than the main bag after thawing.
Hye Ryun Lee,Sue Shin,Sue Shin,Sue Shin,Jong Hyun Yoon,Jong Hyun Yoon,Jong Hyun Yoon,Eun Youn Roh,Eun Youn Roh,Eun Youn Roh,Eun Young Song,Kyou Sup Han,Byoung Jae Kim +12 more
TL;DR: It is concluded that segments have higher CD34+ cells and CFU-GM and lower TNCs than the main cryopreserved bag, although the two compartments are highly correlated with each other.
Journal ArticleDOI
The future of stem cells in liver diseases
TL;DR: In conclusion, while the biological features of stem cells still justify the hope for future clinical applications, hepatic stem cell therapy has still a long way to go from bench to bedside.
Journal ArticleDOI
Analysis of 120 pediatric patients with nonmalignant disorders transplanted using unrelated plasma‐depleted or ‐reduced cord blood
Lawrence Petz,Tang-Her Jaing,Joseph Rosenthal,Chatchada Karanes,Auayporn Nademanee,Lee Lee Chan,M. Graham,Hai Peng Lin,Patrick Tan,B. Wang,Chen Fu,Michelle Chow,Stephen J. Forman,Robert Chow +13 more
TL;DR: Plasma depletion or reduction processing of CB units entails the removal of only a portion of the plasma with minimal nucleated cell loss, however, there are relatively limited data regarding outcomes of CB transplants using units processed by PDR.
Journal ArticleDOI
The history of cord blood transplantation/biology & perspective for future efforts to enhance the field
TL;DR: Efforts are ongoing to modulate cord blood and recipients of cord blood transplantation for more efficacious transplant outcomes, in part in order to overcome the slower time to neutrophil, platelet and immune cell recovery of these compared to the other sources of transplantable cells.
References
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Journal ArticleDOI
Progress in Clinical and Biological Research
TL;DR: All with an interest in tumours will find something to stimulate them in this book but even these items are to some extent outweighed by the insight given into the thoughts and policies of this fascinating country.
Journal ArticleDOI
Human umbilical cord blood as a potential source of transplantable hematopoietic stem/progenitor cells
Hal E. Broxmeyer,Gordon W. Douglas,Giao Hangoc,Scott Cooper,Judith Bard,Denis English,Margaret Arny,Lewis Thomas,Edward A. Boyse +8 more
TL;DR: It was determined that granulocyte-macrophage, erythroid, and multipotential progenitor cells remained functionally viable in cord blood untreated except for addition of anticoagulant for at least 3 days at 4 degrees C or 25 degrees C (room temperature), though not at 37 degrees C, implying that these cells could be satisfactorily studied and used or cryopreserved for therapy.
Journal ArticleDOI
Susceptibility of Fanconi's anaemia fibroblasts to chromosome damage by carcinogens
TL;DR: Experiments in which viable FA fibroblasts were exposed to a direct-acting mutagen or carcinogen for a period of 6 d, ensuring chronic exposure of the cells during one or more cell cycles, until increased cell density inhibited further cell division.
Journal Article
Familial constitutional panmyelocytopathy, Fanconi's anemia (F.A.). I. Clinical aspects.
Journal ArticleDOI
International Fanconi Anemia Registry: relation of clinical symptoms to diepoxybutane sensitivity.
TL;DR: It is concluded that hypersensitivity to the clastogenic effect of DEB is a useful discriminator for FA and a simplified scoring method for classifying patients on the basis of eight clinical manifestations that are the best predictors for FA is presented.