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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Journal ArticleDOI
Stem cells to restore insulin production and cure diabetes
Valeria Sordi,Silvia Pellegrini,M. Krampera,Piero Marchetti,Augusto Pessina,Gianluca Ciardelli,Gian Paolo Fadini,C. Pintus,G. Pantè,Lorenzo Piemonti +9 more
TL;DR: This study focuses on some of the aspects that most frequently raise curiosity and discussion in clinical practice and in the interaction with the patient, and limits the discussion to approaches that have the potential for clinical translation.
Journal ArticleDOI
T lymphocytes of recipient origin may contribute to the recovery of specific immune response toward viruses and fungi in children undergoing cord blood transplantation
Daniela Montagna,Franco Locatelli,Antonia Moretta,Daniela Lisini,Carlo Previderè,Pierangela Grignani,Piero DeStefano,Giovanna Giorgiani,Enrica Montini,Sara Pagani,Patrizia Comoli,Rita Maccario +11 more
TL;DR: It is found that the majority of recipients can develop a specific response to viral or fungal antigens already early after transplantation, and that after CBT recovery of either polyclonal or pauciclonal T-cell response toward widespread pathogens is prompt.
Journal ArticleDOI
Continuous reduced nonrelapse mortality after allogeneic hematopoietic stem cell transplantation: a single-institution's three decade experience.
Florent Malard,Florent Malard,Patrice Chevallier,Thierry Guillaume,Jacques Delaunay,Fanny Rialland,Jean-Luc Harousseau,Philippe Moreau,Francoise Mechinaud,Noel Milpied,Mohamad Mohty +10 more
TL;DR: Data suggest that, despite the fact that older and higher risk patients with more comorbidities underwent transplantation in the last 10 years, NRM decreased while the incidence of relapse increased and the OS improved.
Journal ArticleDOI
Practical placental blood banking.
Paolo Rebulla,Lucilla Lecchi,Laura Porretti,Francesca Poli,Ilaria Ratti,F. Mozzi,Girolamo Sirchia +6 more
TL;DR: The international scientific and lay communities are challenged with the implementation and management of large-scale PB banking programs suitable to provide sufficient numbers of high-quality PB units for the treatment of patients in need of hematopoietic reconstitution.
Journal ArticleDOI
Umbilical Cord Blood: Counselling, Collection, and Banking.
TL;DR: Current evidence regarding umbilical cord blood counselling, collection, and banking is reviewed and guidelines for Canadian health care professionals regarding patient education, informed consent, procedural aspects, and options for cord blood banking in Canada are provided.
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.