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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Umbilical cord blood graft engineering: challenges and opportunities
Philip A. Thompson,Katy Rezvani,Chitra Hosing,Betul Oran,Amanda Olson,Uday R. Popat,Amin M. Alousi,Nina Shah,Simrit Parmar,Catherine M. Bollard,Patrick J. Hanley,Partow Kebriaei,Laurence J.N. Cooper,Joshua Nathan Kellner,Ian McNiece,Elizabeth J. Shpall +15 more
TL;DR: The use of double UCBT rather than single UCBT for most patients is envisaged, which allows for greater ability to treat larger patients as well as to manipulate the graft.
Journal ArticleDOI
Mixed effects of long-term frozen storage on cord tissue stem cells
TL;DR: Poor cell yields and recoveries make frozen CT a less-than-optimal choice for MSC banking, despite good functional recovery, because the amount of fresh CT available at birth is limited and total MSC yields are low.
Journal ArticleDOI
Efficient intracytoplasmic labeling of human umbilical cord blood mesenchymal stromal cells with ferumoxides.
Jae Kwon Lee,Man Kyoung Lee,Hye Jin Jin,Dal-Soo Kim,Yoon Sun Yang,Wonil Oh,Sung-Eun Yang,Tae Seok Park,Soo Yeol Lee,Bum-Soo Kim,Sin-Soo Jeun +10 more
TL;DR: The results suggest the effective concentration for ferumoxides use in tracking MSCs, and suggest that the cytoplasm of adherent cells was effectively labeled at low concentrations of ferum oxides.
Journal Article
Defined serum-free culturing conditions for neural tissue engineering of human cord blood stem cells.
TL;DR: It is shown that cord blood pluripotent stem cells are a potential and promising candidate for future neural tissue engineering and regenerative medicine.
Journal ArticleDOI
The CD34(+)CD38(neg) population is significantly increased in haemopoietic cell expansion cultures in serum-free compared to serum-replete conditions: dissociation of phenotype and function.
TL;DR: Comparison of expansion in serum-replete medium with that inSF conditions demonstrated a lack of expression of CD38 on CD34+ cells in the absence of serum, which must be considered in clinical studies using in vitro expansion in SF conditions, and theCD34+CD38neg phenotype should not be used to confirm maintenance, or expansion, of primitive progenitor cells.
References
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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.
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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.